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Locus control regions, 387
Long interspersed repeat sequences
(LINEs), 321–322
Looped domains, chromatin, 316, 318, 
319f
Loops (protein conformation), 32–33
Loose connective tissue, keratan sulfate I in,
545
Low-density lipoprotein receptor-related
protein, 206
in chylomicron remnant uptake,
208–209, 209f
Low-density lipoproteins, 205, 206t
apolipoproteins of, 206, 206t
metabolism of, 209,210f
ratio of to high-density lipoproteins,
atherosclerosis and, 227
receptors for, 209
in chylomicron remnant uptake,
208–209, 209f
in cotranslational insertion, 505–506,
506f
regulation of, 223
Low-energy phosphates, 83
β-LPH. Seeβ-Lipotropin
LRP. SeeLow-density lipoprotein 
receptor-related protein
L
-tryptophan dioxygenase (tryptophan
pyrrolase), 89
LTs. SeeLeukotrienes
Lung surfactant, 115, 197
deficiency of, 115, 202
Luteinizing hormone (LH), 437, 438, 439f
LXs. SeeLipoxins
LXXLL motifs, nuclear receptor
coregulators, 473
Lyases, 50
in steroid synthesis, 440–442, 441f,
443f
Lymphocyte homing, selectins in, 528–530,
529f,529t,530f
Lymphocytes. See alsoB lymphocytes;
Tlymphocytes
recombinant DNA technology in study
of, 624
Lysine, 16t
catabolism of, 256f,258
pI of, 17
requirements for, 480
Lysine hydroxylase, vitamin C as coenzyme
for, 496
Lysis, cell, complement in, 596
Lysogenic pathway, 379, 379f
Lysolecithin (lysophosphatidylcholine),
116, 116f
metabolism of, 200–201, 201f
Lysophosphatidylcholine. SeeLysolecithin
Lysophospholipase, 200, 201f
Lysophospholipids, 116, 116f
Lysosomal degradation pathway, defect in
in lipidoses, 203
Lysosomal enzymes, 623
in I-cell disease, 431, 432t,531–532,
532f
Lysosomal hydrolases, deficiencies of,
532–533, 533t
Lysosomes
in oligosaccharide processing, 524
protein entry into, 507, 507f,508t
disorders associated with defects in,
512t,513
Lysozyme, 621t
Lysyl hydroxylase
diseases caused by deficiency of, 538t
in hydroxylysine synthesis, 240, 537
Lysyl oxidase, 537, 539
Lytic pathway, 379, 379f
D
-Lyxose, 104f,105t
Mac-1, 529, 529t
α
2
-Macroglobulin, 583t,590, 624
antithrombin activity of, 603
Macromolecules, cellular transport of,
428–431, 429f,430f
Mad cow disease (bovine spongiform
encephalopathy), 37
Magnesium, 496t
in chlorophyll, 270
in extracellular and intracellular fluid,
416, 416t
Major groove, in DNA, 305f,306
operon model and, 378
Malate, 132f,133
Malate dehydrogenase, 132f,133
Malate shuttle, 99, 100f
MALDI. SeeMatrix-assisted laser-desorption
Maleylacetoacetate, in tyrosine catabolism,
254f,255
Malic enzyme, 156t,157
in NADPH production, 176, 176f
Malignancy/malignant cells. See
Cancer/cancer cells
Malignant hyperthermia, 556, 564–565,
565f,569t
Malonate
respiratory chain affected by, 95, 96f
succinate dehydrogenase inhibition by,
67–68, 67f
Malonyl-CoA, in fatty acid synthesis, 173,
174f
Malonyl transacylase, 173, 174f,175f
Maltase, 475
Maltose, 106–107, 107f,107t
Mammalian target of rapamycin (mTOR),
in insulin signal transmission,
466f,467
Mammotropin. SeeProlactin
Manganese, 496t
Mannosamine, 169, 171f
D
-Mannosamine, 106
Mannose, in glycoproteins, 516t
D
-Mannose, 104f,105t
α-
D
-Mannose, 104f
Mannose-binding protein, deficiency of, 533
Mannose 6-phosphate/mannose 6-P signal,
526
in I-cell disease, 531, 532, 532f
in protein flow, 507, 508t
Mannosidosis, 532–533, 533t
MAP (mitogen-activated protein) kinase
in insulin signal transmission, 466f,467
in Jak/STAT pathway, 467
Maple syrup urine disease (branched-chain
ketonuria), 259
Marasmus, 80, 237, 478, 478–479
Marble bone disease (osteopetrosis), 552
Marfan syndrome, fibrillin mutations
causing, 539–540,540f
Maroteaux-Lamy syndrome, 546t
Mass spectrometry, 27, 27f
covalent modifications detected by, 27,
27f,27t
for glycoprotein analysis, 514, 515t
tandem, 27
transcript-protein profiling and, 412
Mast cells, heparin in, 545
Matrix
extracellular, 535–555.See alsospecific
component
mitochondrial, 92, 93f,130
Matrix-assisted laser-desorption (MALDI),
in mass spectrometry, 27
Matrix-processing peptidase, 499
Matrix proteins, 499
diseases caused by defects in import of,
503
Maxam and Gilbert’s method, for DNA
sequencing, 404–405
Maximal velocity (V
max
)
allosteric effects on, 75–76
inhibitors affecting, 68, 68f,69f
Michaelis-Menten equation in
determination of, 65–66, 66f
substrate concentration and, 64, 64f
McArdle’s disease/syndrome, 152t,573
Mechanically gated ion channels, 568t
Mediator-related proteins, 472t,473
Medicine
preventive, biochemical research
affecting, 2
relationship of to biochemistry, 1–4, 3f
Medium-chain acyl-CoA dehydrogenase,
deficiency of, 188
Megaloblastic anemia
folate deficiency causing, 482t,492, 610t
vitamin B
12
deficiency causing, 482t,
492, 494, 610t
Melanocyte-stimulating hormone (MSH),
453, 453f
MELAS (mitochondrial encephalomyopa-
thy with lactic acidosis and
stroke-like episodes), 100–101
672 / INDEX
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Melting point, of amino acids, 18
Melting temperature/transition
temperature, 305, 422
Membrane attack complex, 596
Membrane fatty acid-transport protein, 207
Membrane proteins, 419, 420t,514. See
alsoGlycoproteins
association of with lipid bilayer, 419
flow of, 507,507f,508t
integral, 30, 420, 421f
mutations affecting, diseases caused by,
431–432,432f,432t
peripheral, 420–421, 421f
red cell, 614–617, 615f,616f,616t
structure of, dynamic, 419
Membrane transport, 423, 423t,424f,
426–431,426f.See alsospecific
mechanism
Membranes, 415–433
artificial, 421–422
assembly of, 511–513,512f,512t
asymmetry of, 416, 419–420
bilayers of, 418–419, 418f,419f
membrane protein association and, 419
biogenesis of, 511–513, 512f,512t
cholesterol in, 417
fluid mosaic model and, 422
depolarization of, in nerve impulse
transmission, 428
function of, 415–416,421–422
fluidity affecting, 422
glycosphingolipids in, 417
Golgi apparatus in synthesis of, 509
intracellular, 415
lipids in, 416–418
amphipathic, 119, 120f,417–418, 417f
mutations affecting, diseases caused by,
431–432,432f,432t
phospholipids in, 114–116,115f,
416–417, 417f
plasma. SeePlasma membrane
protein:lipid ratio in, 416, 416f
proteins in, 419, 420t.See also
Membrane proteins
red cell, 614–617,615f,615t,616f,616t
hemolytic anemias and, 619,620t
selectivity of, 415, 423–426,423t,424f,
425f,426t
sterols in, 417
structure of, 416–421,416f
asymmetry and, 416, 419–420
fluid mosaic model of, 421f,422
Menadiol, 486, 487f
Menadiol diacetate, 486, 488f
Menadione, 486. See alsoVitamin K
Menaquinone, 482t,486, 488f.See also
Vitamin K
Menkes disease, 588
MEOS. SeeCytochrome P450-dependent
microsomal ethanol oxidizing
system
6-Mercaptopurine, 290, 291f
Mercapturic acid, 629
Mercuric ions, pyruvate metabolism
affected by, 142
Meromyosin
heavy, 560f,561, 561f
light, 560–561, 560f
Messenger RNA (mRNA), 307, 309–310,
310f,311f,341, 342t,359. See
alsoRNA
alternative splicing and, 354, 354f,
393–394, 636
codon assignments in, 358, 359t
editing of, 356
expression of, detection of in gene
isolation, 635t
modification of, 355–356
nucleotide sequence of, 358
mutations caused by changes in,
361–363,361f,362f,364f
polycistronic, 376
recombinant DNA technology and, 397
relationship of to chromosomal DNA,
321f
stability of, regulation of gene expression
and, 394–395, 394f
transcription starting point and, 342
variations in size/complexity of, 397,
399t
Metabolic acidosis, ammonia in, 245
Metabolic alkalosis, ammonia in, 245
Metabolic fuels, 231–236.See alsoDigestion
clinical aspects of, 236
diet providing, 474, 478
in fed and starving states, 232–234,
233f,234f,234t
interconvertability of, 231–232
Metabolic pathway/metabolite flow, 122,
122–124.See alsospecific type
andMetabolism
flux-generating reactions in, 129
nonequilibrium reactions in, 128–129
regulation of, 72,73f,126–129,128f
covalent modification in, 79
unidirectional nature of, 72, 73f
Metabolism, 81, 122–129,235t.See also
specific type andCatalysis;
Metabolic pathway
blood circulation and, 124–126, 125f,
126f
group transfer reactions in, 8
inborn errors of, 1, 249
integration of, metabolic fuels and,
231–236
regulation of, 72,73f,126–129,128f
allosteric and hormonal mechanisms
in, 74, 74–76,75f,128f,129
enzymes in, 126–129,128f
allosteric regulation and, 74,
74–76,75f,128f,129
compartmentation and, 72–73
control of quantity and, 73–74
covalent modification and, 74, 76,
77–78,78f
rate-limiting reactions and, 73
at subcellular level, 126, 127f
at tissue and organ levels, 124–126, 125f,
126f,235t
of xenobiotics, 626–632
Metachromatic leukodystrophy, 203t
Metal-activated enzymes, 50
Metal ions, in enzymatic reactions, 50
Metalloenzymes, 50
Metalloflavoproteins, 86–87
Metalloproteins, 30
Metallothioneins, 588
Metaphase chromosomes, 317f,318, 319t
Metastasis
glycoproteins and, 514, 526, 530t,531
membrane abnormalities and, 432t
Methacrylyl-CoA, catabolism of, 262f
Methemoglobin, 46, 363, 613–614
Methemoglobinemia, 46, 614
Methionine, 15t,264, 266f
active (S-adenosylmethionine), 258f,
259, 264, 266f,289, 290f,290t
catabolism of, 258f,259, 259f
requirements for, 480
Methionine synthase, 492, 494
Methotrexate, 296–297, 494
dihydrofolate/dihydrofolate reductase
affected by, 296–297, 494
Methylation
in covalent modification, mass increases
and, 27t
of deoxycytidine residues, gene
expression affected by, 383
in glycoprotein analysis, 515t
of xenobiotics, 626, 630
β-Methylcrotonyl-CoA, catabolism of, 261f
5-Methylcytosine, 287, 289f
α-Methyldopa, 446
Methylene tetrahydrofolate, 493, 493f
in folate trap, 493f,494
7-Methylguanine, 289f
Methylhistidine, 576
in Wilson’s disease, 265
Methylmalonic aciduria, 155
Methylmalonyl-CoA, accumulation of in
vitamin B
12
deficiency, 492
Methylmalonyl-CoA isomerase (mutase), in
propionate metabolism, 155,
155f,492
Methylmalonyl-CoA mutase (isomerase),
155, 155f,492
Methylmalonyl-CoA racemase, in propi-
onate metabolism, 155, 155f
Methyl pentose, in glycoproteins, 109t
Methyl-tetrahydrofolate, in folate trap,
493f,494
Mevalonate, synthesis of, in cholesterol
synthesis, 219, 220f,221f,222f
INDEX / 673
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Mg. SeeMagnesium
Micelles, 418, 418f
amphipathic lipids forming, 119, 120f,
418, 418f
in lipid absorption, 475
Michaelis constant (K
m
), 65
allosteric effects on, 75–76
binding constant approximated by, 66
enzymatic catalysis rate and, 65–66, 66f,
72, 73f
inhibitors affecting, 68, 69f
Michaelis-Menten equation in
determination of, 65–66, 66f
Michaelis-Menten equation, 65
Bi-Bi reactions and, 70, 70f
regulation of metabolite flow and, 72,
73f
Microfilaments, 576–577
α
2
-Microglobulin, 583t
Microsatellite instability, 322
Microsatellite polymorphism, 322, 411,
413
Microsatellite repeat sequences, 322, 413
Microsomal elongase system, 177, 177f
Microsomal fraction, cytochrome P450
isoforms in, 627
Microtubules, 577
Migration, cell, fibronectin in, 540
Milk (lactose) intolerance, 102, 474, 475
Mineralocorticoid response element, 459t
Mineralocorticoids, 437
receptor for, 471
synthesis of, 438–440, 441f
Minerals, 2, 496–497,496t
digestion and absorption of, 477–478
Minor groove, in DNA, 305f,306
Mismatch repair of DNA, 336, 336f,336t
colon cancer and, 336
Missense mutations, 361, 362–363, 362f
familial hypertrophic cardiomyopathy
caused by, 569–570, 570f
MIT. SeeMonoiodotyrosine
Mitchell’s chemiosmotic theory. See
Chemiosmotic theory
Mitochondria
ALA synthesis in, 270, 273f
citric acid cycle in, 122, 122f,123f,124f,
126, 127f,130, 133–135,134f
fatty acid oxidation in, 180–181,181f
ion transport in, 99
protein synthesis and import by,
499–501,501t
respiration rate of, ADP in control of,
94–95, 97t,98f
respiratory chain in, 92. See also
Respiratory chain
Mitochondrial cytochrome P450, 89–90,
627. See alsoCytochrome P450
system
Mitochondrial DNA, 322–323,322f,
323t
Mitochondrial encephalomyopathies, with
lactic acidosis and stroke-like
episodes (MELAS), 100
Mitochondrial genome, 499
Mitochondrial glycerol-3-phosphate
dehydrogenase, 87
Mitochondrial membrane proteins, muta-
tions of, 431
Mitochondrial membranes, 92, 93f
enzymes as markers and, 92
exchange transporters and, 98–100,98f,
99f
protein insertion in, 501
Mitochondrial myopathies, fatal infantile,
and renal dysfunction, oxidore-
ductase deficiency causing, 100
Mitogen-activated protein (MAP) kinase
in insulin signal transmission, 466f,
467
in Jak/STAT pathway, 467
Mitotic spindle, microtubules in formation
of, 577
Mixed-function oxidases, 89–90, 627. See
alsoCytochrome P450 system
ML. SeeMucolipidoses
MOAT. SeeMultispecific organic anion
transporter
Modeling, molecular, in protein structure
analysis, 36
Molecular biology, 1. See alsoRecombinant
DNA/recombinant DNA
technology
in primary structure determination,
25–26
Molecular chaperones. SeeChaperones
Molecular genetics, 1, 396. See also
Recombinant DNA/recombinant
DNA technology
Molecular modeling, in protein structure
analysis, 36
Molecular motors, 577
Molybdenum, 496t
Monoacylglycerol acyltransferase, 198f,199
Monoacylglycerol pathway, 198f,199,
475–477, 476f
2-Monoacylglycerols, 198f,199
Monoclonal antibodies, hybridomas in
production of, 595–596, 596f
Monoglycosylated core structure, calnexin
binding and, 526
Monoiodotyrosine (MIT), 447, 448f,449
Monomeric proteins, 34
Mononucleotides, 287
“salvage” reactions and, 294,295f,297f
Monooxygenases, 89–90. See also
Cytochrome P450 system
in metabolism of xenobiotics, 626
Monosaccharides, 102. See alsospecific type
andGlucose
absorption of, 475, 475f
physiologic importance of, 104–105, 105t
Monounsaturated fatty acids, 112, 113t.See
alsoFatty acids; Unsaturated fatty
acids
dietary, cholesterol levels affected by, 227
synthesis of, 191,191f
Morquio syndrome, 546t
MPP. SeeMatrix-processing peptidase
MPS. SeeMucopolysaccharidoses
MRE. SeeMineralocorticoid response
element
mRNA. SeeMessenger RNA
MRP2. SeeMultidrug resistance-like
protein 2
MSH. SeeMelanocyte-stimulating hormone
MstII,399t
in sickle cell disease, 409, 410f
mtDNA. SeeMitochondrial DNA
mTOR, in insulin signal transmission,
466f,467
Mucins, 519–520, 520t
genes for, 520
O-glycosidic linkages in, 518, 519–520,
519f
repeating amino acid sequences in, 519,
520f
Mucolipidoses, 546–547, 546t
Mucopolysaccharides, 109, 109f
Mucopolysaccharidoses, 545–547, 546t,
547f
Mucoproteins. SeeGlycoproteins
Mucus, 519–520
Multidrug resistance-like protein 2, in
bilirubin secretion, 280
Multipass membrane protein, anion
exchange protein as, 615, 615f,
616t
Multiple myeloma, 595
Multiple sclerosis, 202
Multiple sulfatase deficiency, 203
Multisite phosphorylation, in glycogen
metabolism, 151
Multispecific organic anion transporter, in
bilirubin secretion, 280
Muscle, 556–576,557f.See alsoCardiac
muscle; Skeletal muscle
ATP in, 556, 561–562, 573–574,575f
contraction of. SeeMuscle contraction
in energy transduction, 556–559,557f,
558f,559f
fibers in, 556
glycogen in, 145, 146t
metabolism in, 125, 125f,235t,576t
glycogen, 145
lactate production and, 139
as protein reserve, 576
proteins of, 566t.See alsoActin; Myosin;
Titin
Muscle contraction, 556, 558f,561–565,
564t
ATP hydrolysis in, 561–562, 561f
in cardiac muscle, 566–568
674 / INDEX
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regulation of
actin-based, 562–563
calcium in, 562
in cardiac muscle, 566–568
sarcoplasmic reticulum and,
563–564, 563f,564f
in smooth muscle, 570–571, 571f
myosin-based, 570
myosin light chain kinase in,
570–571, 571f
relaxation phase of, 561, 564, 564t
in smooth muscle
calcium in, 571
nitric oxide in, 571–573, 573f
sliding filament cross-bridge model of,
557–559, 558f
in smooth muscle, 570–573
tropomyosin and troponin in, 562
Muscle fatigue, 136
Muscle phosphorylase, 147
absence of, 152t
activation of
calcium/muscle contraction and, 
148
cAMP and, 147–148, 149f
Muscular dystrophy, Duchenne, 556,
565–566,566f
Mutagenesis, site-directed, in enzyme study,
58
Mutations, 314, 323–326,323f,324f,
325f
base substitution, 361, 361f,362
constitutive, 376
frameshift, 363, 364f
ABO blood group and, 619
gene conversion and, 325
integration and, 324, 324f
of membrane proteins, diseases caused
by, 431–432,432f,432t
missense, 361, 362–363, 362f
familial hypertrophic cardiomyopathy
caused by, 569–570, 570f
mRNA nucleotide sequence changes caus-
ing, 361–363,361f,362f,364f
nonsense, 362
point, 361
recombinant DNA technology in
detection of, 408–409, 408f
recombination and, 323–324, 323f,324f
silent, 361
sister chromatid exchanges and, 325, 325f
suppressor, 363
transition, 361, 361f
transposition and, 324–325
transversion, 361, 361f
Myasthenia gravis, 431
Myelin sheets, 428
Myeloma, 595
Myeloma cells, hybridomas grown from,
596, 596f
Myeloperoxidase, 612, 621t,623
Myocardial infarction, lactate
dehydrogenase isoenzymes in
diagnosis of, 57, 57t,58f
Myofibrils, 556, 557f,558f
Myoglobin, 40–48
α-helical regions of, 40, 41f
βsubunits of hemoglobin and, 42
oxygen dissociation curve for, 41–42, 42f
oxygen stored by, 40, 41–42, 42f,573
Myoglobinuria, 47
Myokinase (adenylyl kinase), 84
deficiencies of, 151–152
in gluconeogenesis regulation, 157
as source of ATP in muscle, 573, 575f
Myopathies, 92
mitochondrial, 100–101
fatal infantile, and renal dysfunction,
oxidoreductase deficiency
causing, 100
Myophosphorylase deficiency, 152t
Myosin, 557, 559,560f
in muscle contraction, 557–559, 558f,
561–562, 561f,562f
regulation of smooth muscle
contraction and, 570
in striated versus smooth muscle, 572t
structure and function of, 560–561, 560f
Myosin-binding protein C, 566t
Myosin (thick) filaments, 557, 558f
Myosin head, 560, 560f
conformational changes in, in muscle
contraction, 561
Myosin heavy chains, 560
familial hypertrophic cardiomyopathy
caused by mutations in gene for,
569–570, 570f
Myosin light chain kinase, 570–571, 571f
Myosin light chains, 560
in smooth muscle contraction, 570
Myotonia congenita, 569t
Myristic acid, 112t,510
Myristylation, 510
in covalent modification, mass increases
and, 27t
N-acetyl neuraminic acid, 169, 171f
in gangliosides, 201, 203f
in glycoproteins, 169, 171f,515, 516t
in mucins, 519f,520
N-linked glycoproteins, 518, 519f,
521–527
classes of, 521, 522f
synthesis of, 521–527, 523f,524f,525f,
526t
dolichol-P-P-oligosaccharide in,
521–524, 523f
in endoplasmic reticulum and Golgi
apparatus, 524–525, 526t
glycan intermediates formed during,
526
regulation of, 526–527, 527t
tunicamycin affecting, 527, 527t
Na. SeeSodium
Na+-Ca2+exchanger, 463
Na
+
-K
+
ATPase, 427–428, 428f
in glucose transport, 428, 429f
NAD(nicotinamide adenine dinucleotide),
87, 490, 490f
absorption spectrum of, 56, 56f
in citric acid cycle, 133
as coenzyme, 87, 89f,290t
NADH
absorption spectrum of, 56, 56f
extramitochondrial, oxidation of, 99,
100f
fatty acid oxidation yielding, 181
in pyruvate dehydrogenase regulation,
141–142, 142f
NADH dehydrogenase, 87, 93
NADP
+
(nicotinamide adenine dinucleotide
phosphate), 87, 490
as coenzyme, 87, 89f,290t
in pentose phosphate pathway, 163,
164f,165f
NAD(P)+-dependent dehydrogenases, in
enzyme detection, 56
NADPH
in cytochrome P450 reactions, 90f,627
intramitochondrial, proton-translocating
transhydrogenase and, 99
for lipogenesis, 175f,176, 176f
pentose phosphate pathway and, 163,
164f,165f,169
NADPH-cytochrome P450 reductase, 627
NADPH oxidase, 621t,622–623
chronic granulomatous disease associated
with mutations in, 623, 623f
NCoA-1/NCoA-2 coactivators, 472, 472t
NCoR, 472t,473
NDPs. SeeRibonucleoside diphosphates
Nebulin, 566t
NEFA (nonesterified fatty acids). SeeFree
fatty acids
Negative nitrogen balance, 479
Negative regulators, of gene expression,
374, 375t,378, 380
Negative supercoils, DNA, 306
NEM-sensitive factor (NSF), 509, 510f
Neonatal adrenoleukodystrophy, 503, 
503t
Neonatal (physiologic) jaundice, 282–283
Neonatal tyrosinemia, 255
Neonate, hypoglycemia in, 151
Nerve cells. SeeNeurons
Nerve impulses, 428
Nervous system
glucose as metabolic necessity for, 232
thiamin deficiency affecting, 489
NESs. SeeNuclear export signals
Net charge, of amino acid, 16–17, 17f
Net diffusion, 423
INDEX / 675
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NeuAc. SeeN-Acetylneuraminic acid
Neural tube defects, folic acid supplements
in prevention of, 494
Neuraminic acid, 110, 116
Neuraminidases
deficiency of, 532–533, 533t
in glycoprotein analysis, 517
influenza virus, 533
Neurofilaments, 577t
Neurologic diseases, protein conformation
alterations and, 37
Neurons, membranes of
impulses transmitted along, 428
ion channels in, 424, 425f
synaptic vesicle fusion with, 511
Neuropathy, sensory, in vitamin B
6
excess,
491
Neutral lipids, 111
Neutropenia, 610
Neutrophils, 620–624
activation of, 621–622
biochemical features of, 620t
enzymes and proteins of, 621t
in infection, 620
in inflammation, 620, 621t
integrins and, 620–621, 622t
selectins and, 528–529, 529t,530f
proteinases of, 623–624, 624t
respiratory burst and, 622–623
NF-κB pathway, 468, 468f,469f
Niacin, 482t,490,490f.See also
Nicotinamide; Nicotinic acid
in citric acid cycle, 133
deficiency of, 482t,490
excess/toxicity of, 490
Nick translation, 413
Nickel, 496t
Nicks/nick-sealing, in DNA replication,
332, 332f
Nicotinamide, 482t,490, 490f.See also
Niacin
coenzymes derived from, 50–51
dehydrogenases and, 87, 89f
excess/toxicity of, 490
Nicotinamide adenine dinucleotide
(NAD+), 87, 490, 490f
absorption spectrum of, 56, 56f
in citric acid cycle, 133
as coenzyme, 87, 89f,290t
Nicotinamide adenine dinucleotide
phosphate (NADP+), 87, 490
as coenzyme, 87, 89f,290t
in pentose phosphate pathway, 163,
164f,165f
Nicotinic acid, 482t,490, 490f.See also
Niacin
as hypolipidemic drug, 229
NIDDM. SeeNon-insulin dependent
diabetes mellitus
Nidogen (entactin), in basal lamina, 540
Niemann-Pick disease, 203t
Night blindness, vitamin A deficiency
causing, 482t,483
Nitric oxide, 556, 571–573, 573f,574t,
607t
clotting/thrombosis affected by, 607,
607t
Nitric oxide synthases, 572–573, 573f,574t
Nitrite, nitric oxide formation from, 572
Nitrogen, amino acid (α-amino)
catabolism of, 242–248
end products of, 242–243
urea as, 242–243, 245–247,246f
L
-glutamate dehydrogenase in,
244–245,244f,245f
Nitrogen balance, 479
Nitroglycerin, 572
NLS. SeeNuclear localization signal
NMR. SeeNuclear magnetic resonance
(NMR) spectroscopy
NO. SeeNitric oxide
NO synthase. SeeNitric oxide synthase
Noncoding regions, in recombinant DNA
technology, 397, 398f
Noncoding strand, 304
Noncompetitive inhibition, competitive
inhibition differentiated from,
67–69,67f,68f,69f
Noncovalent assemblies, in membranes, 416
Noncovalent forces
in biomolecule stabilization, 6
peptide conformation and, 20
Nonequilibrium reactions, 128–129
citric acid cycle regulation and, 135
glycolysis regulation and, 140, 153–155
Nonesterified fatty acids. SeeFree fatty acids
Nonfunctional plasma enzymes, 57. See also
Enzymes
in diagnosis and prognosis, 57, 57t
Nonheme iron, 92, 95f,585
Nonhistone proteins, 314
Non-insulin dependent diabetes mellitus
(NIDDM/type 2), 161
Nonoxidative phase, of pentose phosphate
pathway, 163–166
Nonrepetitive (unique-sequence) DNA,
320, 320–321
Nonsense codons, 359, 361, 363
Nonsense mutations, 361
Nonsteroidal anti-inflammatory drugs
cyclooxygenase affected by, 193
prostaglandins affected by, 190
Norepinephrine, 439f,447, 447f.See also
Catecholamines
synthesis of, 267, 267f,445–447, 447f
in thermogenesis, 217, 217f
Northern blot transfer procedure, 305–306,
403, 404f,413
in gene isolation, 635t
NPCs. SeeNuclear pore complexes
NSF. SeeNEM-sensitive factor
Nuclear export signals, 503
Nuclear genes, proteins encoded by, 499
Nuclear localization signal (NLS), 501,
502f,508t
Nuclear magnetic resonance (NMR)
spectroscopy
for glycoprotein analysis, 514, 515t
protein structure demonstrated by,
35–36
Nuclear pore complexes, 501
Nuclear proteins, O-glycosidic linkages in,
518
Nuclear receptor coactivators 
(NCoA-1/NCoA-2), 472, 472t
Nuclear receptor corepressor (NCoR), 472t,
473
Nuclear receptor superfamily, 436, 469,
469–471, 471f,472t
Nucleases, 8, 312
active chromatin and, 316
Nucleic acids. See alsoDNA; RNA
bases of, 287–289, 288t
dietarily nonessential, 293
digestion of, 312
structure and function of, 303–313
Nucleolytic processing, of RNA, 352
Nucleophile, water as, 7–8
Nucleophilic attack, in DNA synthesis,
328, 329f
Nucleoplasmin, 315
Nucleoproteins, packing of, 318, 319t,320f
Nucleosidases (nucleoside phosphorylases),
purine, deficiency of, 300
Nucleoside diphosphate kinase, 85
Nucleoside triphosphates
group transfer potential of, 289–290,
289f,290f,290t
nonhydrolyzable analogs of, 291, 292f
in phosphorylation, 85
Nucleosides, 286–287, 288t
Nucleosomes, 314, 315–316, 315f
Nucleotide excision-repair of DNA, 336,
337, 338f
Nucleotide sugars, in glycoprotein biosyn-
thesis, 516–517,516t,520, 521t
Nucleotides, 286–292,288t.See also
Purine; Pyrimidines
adenylyl kinase (myokinase) in
interconversion of, 84
as coenzymes, 290, 290t
DNA, deletion/insertion of, frameshift
mutations and, 363, 364f
metabolism of, 293–302
in mRNA, 358
mutations caused by changes in,
361–363,361f,362f,364f
physiologic functions of, 289
as polyfunctional acids, 290
polynucleotides, 291–292
synthetic analogs of, in chemotherapy,
290–291,291f
ultraviolet light absorbed by, 290
676 / INDEX
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Nucleus (cell), importins and exportins in
transport and, 501–503,502f
Nutrition, 474–480.See alsoDiet
biochemical research affecting, 2
lipogenesis regulated by, 177–178
Nutritional deficiencies, 474
in AIDS and cancer, 479
Nutritionally essential amino acids, 124,
237t,480. See alsoAmino acids
Nutritionally essential fatty acids, 190.See
alsoFatty acids
abnormal metabolism of, 195–196
deficiency of, 191–192, 194–195
Nutritionally nonessential amino acids,
124, 237, 237t,480
synthesis of, 237–241
O
R
SeeRight operator
O blood group substance, 618–619, 619f
Ogene, 618–619
O-glycosidic linkage
of collagen, 537
of proteoglycans, 542–543
O-linked glycoproteins, 518, 518–520,
519f,520f,520t
synthesis of, 520, 521t
O-linked oligosaccharides, in mucins,
519–520, 519f,520f
Obesity, 80, 205, 231, 474, 478
lipogenesis and, 173
Octamers, histone, 315, 315f
Oculocerebrorenal syndrome, 512t
1,25(OH)
2
-D
3
SeeCalcitriol
3β-OHSD. See3β-Hydroxysteroid
dehydrogenase
Okazaki fragments, 327, 330, 331f
Oleic acid, 112, 112f,113, 113t,114f,190f
synthesis of, 191, 191f
Oligomers, import of by peroxisomes, 503
Oligomycin, respiratory chain affected by,
95, 96f,97f
Oligonucleotide
definition of, 413
in primary structure determination, 26
Oligosaccharide:protein transferase, 523
Oligosaccharide branches (antennae), 521
Oligosaccharide chains
glycoprotein, 514,515t,581–582
in N-glycosylation, 524, 525f
regulation of, 526
sugars in, 515,516t
glycosaminoglycans, 543
Oligosaccharide processing, 521, 524, 525f
Golgi apparatus in, 509
regulation of, 526, 527f
Oligosaccharides, 102
O-linked, in mucins, 519–520, 519f,
520f
OMP (orotidine monophosphate), 296,
298f
Oncogenes, 1
cyclins and, 334
Oncoproteins, Rb protein and, 334
Oncotic (osmotic) pressure, 580, 584
Oncoviruses, cyclins and, 334
Open complex, 345
Operator locus, 377–378, 377f,378
Operon/operon hypothesis, 375, 376–378,
376f,377f
Optical activity/isomer, 104
ORC. SeeOrigin replication complex
ORE. SeeOrigin replication element
Ori (origin of replication), 326, 327f,413
Origin replication complex, 326
Origin replication element, 326
Origin of replication (ori), 326, 327f,
413
Ornithine, 265, 266f
catabolism of, 250, 251f
in urea synthesis, 245, 246–247, 246f,
247
Ornithine δ-aminotransferase, mutations
in, 250
Ornithine-citrulline antiporter, defective,
250
Ornithine transcarbamoylase/
L
-Ornithine
transcarbamoylase
deficiency of, 247, 300
in urea synthesis, 246–247, 246f
Orosomucoid (α
1
-acid glycoprotein), 
583t
Orotate phosphoribosyltransferase, 296,
297, 298f
Orotic aciduria, 300, 301
Orotidine monophosphate (OMP), 296,
298f
Orotidinuria, 301
Orphan receptors, 436, 471
Osmotic fragility test, 617
Osmotic lysis, complement in, 596
Osmotic (oncotic) pressure, 580, 584
Osteoarthritis, 535, 551t
proteoglycans in, 548
Osteoblasts, 549, 549f,550
Osteocalcin, 488, 496, 548t
Osteoclasts, 549–550, 549f,550f
Osteocytes, 549, 549f
Osteogenesis imperfecta (brittle bones),
551–552, 551t
Osteoid, 549f,550
Osteomalacia, 482t,484, 485, 551t
Osteonectin, 548t
Osteopetrosis (marble bone disease), 552
Osteoporosis, 485, 551t,552
Osteopontin, 548t
Ouabain, 106
Na+-K+ATPase affected by, 428
Outer mitochondrial membrane, 92, 93f
protein insertion in, 501
Ovary, hormones produced by, 437,
442–445, 444f,445f
Overnutrition, 478–479
Oxaloacetate
in amino acid carbon skeleton
catabolism, 249, 250f
in aspartate synthesis, 237–238, 238f
in citric acid cycle, 126, 127f,130, 131f,
133, 134f,135
Oxalosis, 170
Oxidases, 86, 86–87,87f.See alsospecific
type
ceruloplasmin as, 587
copper in, 86
flavoproteins as, 86–87, 88f
mixed-function, 89–90, 627. See also
Cytochrome P450 system
Oxidation, 86–91
definition of, 86
dehydrogenases in, 87–88,88f,89f
fatty acid, 180–189.See alsoKetogenesis
acetyl-CoA release and, 123–124,
123f,181–183,181f,182f
β, 181–183,181f,182f
ketogenesis regulation and,
186–187, 187f,188f
modified, 183,183f
clinical aspects of, 187–189
hypoglycemia caused by impairment
of, 187–188
in mitochondria, 180–181,181f
hydroperoxidases in, 88–89
oxidases in, 86–87,87f,88f
oxygen toxicity and, 90–91,611–613,
613t
oxygenases in, 89–90,90f
redox potential and, 86,87t
Oxidation-reduction (redox) potential, 86,
87t
Oxidative decarboxylation, of 
α-ketoglutarate, 131, 132f
Oxidative phase, of pentose phosphate
pathway, 163, 164f,165f
Oxidative phosphorylation, 83, 92–101,
122. See alsoPhosphorylation;
Respiratory chain
chemiosmotic theory of, 92, 95–97,97f
clinical aspects of, 100–101
muscle generation of ATP by, 573,
574–576, 575f,575t
poisons affecting, 92, 95,96f
Oxidative stress, 612
Oxidoreductases, 49, 86. See alsospecific type
deficiency of, 100
Oxidosqualene:lanosterol cyclase, 220, 222f
Oxygen
binding, 42, 42f.See alsoOxygenation
Bohr effect and, 44, 45f
histidines F8 and E7 in, 40, 41f
hemoglobin affinities (P
50
) for, 42–43,
43f
myoglobin in storage of, 40, 41–42, 42f,
573
INDEX / 677
Oxygen (cont.)
reductive activation of, 627
transport of, ferrous iron in, 40–41
Oxygen dissociation curve, for myoglobin
and hemoglobin, 41–42,42f
Oxygen radicals. SeeFree radicals
Oxygen toxicity, superoxide free radical
and, 90–91, 611–613, 613t.See
alsoFree radicals
Oxygenases, 86, 89–90
Oxygenation of hemoglobin
conformational changes and, 42, 43f,44f
apoprotein, 42
2,3-bisphosphoglycerate stabilizing,
45, 45f
high altitude adaptation and, 46
mutant hemoglobins and, 46
Oxysterols, 119
P
i
, in muscle contraction, 561, 561f
P
50
, hemoglobin affinity for oxygen and,
42–43, 43f
p53 protein/p53gene, 339
p160 coactivators, 472, 472t
p300 coactivator/CPB/p300, 461, 468,
469, 469f,472, 472t
P450 cytochrome. SeeCytochrome P450
system
P450scc (cytochrome P450 side chain
cleavage enzyme), 438, 440f,442
p/CIP coactivator, 472, 472t
P component, in amyloidosis, 590
P-selectin, 529t
PAC (P1-based) vector, 401–402, 402t,413
PAF. SeePlatelet-activating factor
PAGE. SeePolyacrylamide gel
electrophoresis
Pain, prostaglandins in, 190
Palindrome, 413
Palmitate, 173, 173–174
Palmitic acid, 112t
Palmitoleic acid, 113t,190f
Palmitoylation, in covalent modification,
mass increases and, 27t
Pancreatic insufficiency, in vitamin B
12
deficiency, 492
Pancreatic islets, insulin produced by, 160
Pancreatic lipase, 475, 476f
Panproteinase inhibitor, α
2
-macroglobulin
as, 590
Pantothenic acid, 173, 482t,495, 495i
in citric acid cycle, 133
coenzymes derived from, 51
deficiency of, 482t
Papain, immunoglobulin digestion by, 591
PAPS. SeeAdenosine 3′-phosphate-
5′-phosphosulfate
Parallel βsheet, 32, 33f
Parathyroid hormone (PTH), 438, 450,
451f
storage/secretion of, 453, 454t
synthesis of, 450, 451f
Paroxysmal nocturnal hemoglobinuria,
432t,528, 530t,531, 531f
Partition chromatography, for protein/
peptide purification, 21
Passive diffusion/transport, 423, 423t,424f
Pasteur effect, 157
pBR322, 402, 402t,403f
PCR. SeePolymerase chain reaction
PDH. SeePyruvate dehydrogenase
PDI. SeeProtein disulfide isomerase
PECAM-1, 529, 529t
Pedigree analysis, 409, 410f
Pellagra, 482t,490
Penicillamine, for Wilson disease, 589
Pentasaccharide, in N-linked glycoproteins,
521, 522f
Pentose phosphate pathway, 123, 163–166,
164f,165f,167f
cytosol as location for reactions of, 163
enzymes of, 156t
erythrocyte hemolysis and, 169–170, 
613
impairment of, 169–170
NADPH produced by, 163,164f,
165f
for lipogenesis, 175f,176, 176f
nonoxidative phase of, 163–166
oxidative phase of, 163, 164f,165f
ribose produced by, 163,164f
Pentoses, 102, 102t
in glycoproteins, 109t
physiologic importance of, 104–105,
105t
Pentosuria, essential, 163, 170
PEPCK. SeePhosphoenolpyruvate
carboxykinase
Pepsin, 477
in acid-base catalysis, 52
Pepsinogen, 477
Peptidases, in protein degradation, 242,
243f
Peptide bonds. See alsoPeptides
formation of, 7, 368
partial double-bond character of, 19–20,
20f
Peptides, 14–20,439f.See alsoAmino
acids; Proteins
absorption of, 477
amino acids in, 14, 19, 19f
formation of, 
L
-α-amino acids in, 14
as hormone precursors, 449–453
intracellular messengers used by,
457–468,461t,463t
as polyelectrolytes, 19
purification of, 21–24
Peptidyl prolyl isomerase, 508
Peptidylglycine hydroxylase, vitamin C as
coenzyme for, 496
Peptidyltransferase, 368, 370t
Periodic acid-Schiff reagent, in glycoprotein
analysis, 515t
Periodic hyperlysinemia, 258
Periodic paralysis
hyperkalemic, 569t
hypokalemic, 569t
Peripheral proteins, 420–421, 421f
Peripherin, 577t
Permeability coefficients, of substances in
lipid bilayer, 418, 419f
Pernicious anemia, 482t,492
Peroxidases, 88, 192
Peroxidation, lipid, free radicals produced
by, 118–119,120f
Peroxins, 503
Peroxisomal-matrix targeting sequences
(PTS), 503, 508t
Peroxisomes, 89, 503
absence/abnormalities of, 503, 503t
in Zellweger’s syndrome, 188, 503
biogenesis of, 503
in fatty acid oxidation, 182–183
Pfeiffer syndrome, 551t
PFK-1. SeePhosphofructokinase
PGHS. SeeProstaglandin H synthase
PGIs. SeeProstacyclins
PGs. SeeProstaglandins
pH, 9–13.See alsoAcid-base balance
amino acid net charge and, 16, 17f
buffering and, 11–12, 12f.See also
Buffers
calculation of, 9–10
definition of, 9
enzyme-catalyzed reaction rate affected
by, 64, 64f
isoelectric, amino acid net charge and, 17
Phage lambda, 378–383, 379f,380f,381f,
382f
Phages
for cloning in gene isolation, 635t
in recombinant DNA technology, 401
Phagocytic cells, respiratory burst of,
622–623
Phagocytosis, 429
Pharmacogenetics, 630, 631–632
Pharmacogenomics, 632, 638
Phasing, nucleosome, 315–316
Phenobarbital, warfarin interaction and,
cytochrome P450 induction
affecting, 628
Phenylalanine, 16t
catabolism of, 255–258, 255f
in phenylketonuria, 255, 255f
requirements for, 480
in tyrosine synthesis, 239, 240f
Phenylalanine hydroxylase
defect in, 255
localization of gene for, 407t
in tyrosine synthesis, 239, 240f
Phenylethanolamine-N-methyltransferase
(PNMT), 447, 447f
678 / INDEX
Phenylisothiocyanate (Edman reagent), in
protein sequencing, 25, 26f
Phenylketonuria, 255–258
Phi (ϕ) angle, 31, 31f
Phosphagens, 83, 84f
Phosphatases
acid, diagnostic significance of, 57t
alkaline
in bone mineralization, 550
isozymes of, diagnostic significance of,
57t
in recombinant DNA technology,
400t
Phosphate transporter, 99, 99f
Phosphates/phosphorus, 496t
exchange transporters and, 99, 99f,100,
101f
in extracellular and intracellular fluid,
416t
free energy of hydrolysis of, 82–83, 82t
high-energy, 83. See alsoATP
in energy capture and transfer, 82–83,
82f,82t,83f
as “energy currency” of cell, 83–85,
84f,85f
symbol designating, 83
transport of, creatine phosphate
shuttle in, 100, 101f
low-energy, 83
Phosphatidate, 198f,199
in triacylglycerol synthesis, 197, 197f,
198, 198f,199
Phosphatidate phosphohydrolase, 198f,199
Phosphatidic acid, 114, 115f,416–417,
417f
Phosphatidic acid pathway, 476f,477
Phosphatidylcholines (lecithins), 114–115,
115f
in cytochrome P450 system, 617
membrane asymmetry and, 420
synthesis of, 197, 197f,198f
Phosphatidylethanolamine (cephalin), 115,
115f
membrane asymmetry and, 420
synthesis of, 197, 197f
Phosphatidylglycerol, 115, 115f
Phosphatidylinositol/phosphatidylinositide,
115, 115f
in blood coagulation, 601
GPI-linked glycoproteins and, 527. 
See alsoGlycosylphosphatidyli-
nositol-anchored (GPI-
anchored/ GPI-linked) 
glycoproteins
metabolism of, 464–465, 464f,465f
as second messenger/second messenger
precursor, 115, 115f,437, 437t,
457, 463–465, 463t,464f,465f
synthesis of, 197, 197f,198f
Phosphatidylinositol 4,5-bisphosphate, 115,
464–465, 465f
in neutrophil activation, 621–622
in platelet activation, 606–607, 606f
Phosphatidylinositol 3-kinase (PI-3 kinase)
in insulin signal transmission, 465, 466f
in Jak/STAT pathway, 467
Phosphatidylserine, 115, 115f
in blood coagulation, 601
membrane asymmetry and, 420
Phosphocreatine, in muscle, 556
Phosphodiester, 291
Phosphodiesterases, 291
in calcium-dependent signal
transduction, 463
in cAMP-dependent signal transduction,
461, 462f
cAMP hydrolyzed by, 147
Phosphoenolpyruvate, 156t
free energy of hydrolysis of, 82t
in gluconeogenesis, 133, 134f,156t
Phosphoenolpyruvate carboxykinase
(PEPCK), 133, 134f
in gluconeogenesis regulation, 133, 134f,
153, 154f
Phosphofructokinase/
phosphofructokinase-1, 156t
in gluconeogenesis regulation, 157
in glycolysis, 137, 138f,156t
regulation and, 140
muscle, deficiency of, 143, 152t
Phosphofructokinase-2, 157, 158f
Phosphoglucomutase, in glycogen
biosynthesis, 145, 146f
6-Phosphogluconate dehydrogenase, 156t,
163, 164f,165f
3-Phosphoglycerate
in glycolysis, 137, 138f
in serine synthesis, 238, 238f
Phosphoglycerate kinase, in glycolysis, 137,
138f
in erythrocytes, 140, 140f
Phosphoglycerate mutase, in glycolysis, 137,
138f
Phosphoglycerides, in membranes,
416–417, 417f
Phosphoglycerols
lysophospholipids in metabolism of, 116,
116f
synthesis of, 197f,198f,199
Phosphohexoseisomerase, in glycolysis, 137,
138f
Phosphoinositide-dependent kinase-1
(PDK1), in insulin signal
transmission, 465
Phospholipase A
1
, 200, 201f
Phospholipase A
2
, 200, 201f
in platelet activation, 606f,607
Phospholipase C, 200, 201f
in calcium-dependent signal transduction,
464–465, 464f,465f
in Jak/STAT pathway, 467
in respiratory burst, 623
Phospholipase Cβ, in platelet activation,
606, 606f
Phospholipase D, 200, 201f
Phospholipases
in glycoprotein analysis, 515t
in phosphoglycerol degradation and
remodeling, 200–201, 201f
Phospholipids, 111, 205
digestion and absorption of, 475–477,
476f
glycerol ether, synthesis of, 199, 200f
in lipoprotein lipase activity, 207–208
in membranes, 114–116,115f,
416–417, 417f,419, 511
membrane asymmetry and, 420, 511
in multiple sclerosis, 202
as second messenger precursors, 197
synthesis of, 198f
Phosphoprotein phosphatases, in cAMP-
dependent signal transduction,
462, 462f
Phosphoproteins, in cAMP-dependent
signal transduction, 461, 462f
Phosphoric acid, pK/pK
a
value of, 12t
Phosphorus. SeePhosphates
Phosphorylase
in glycogen metabolism, 145–146, 
146f
regulation of, 148–150, 150–151,
150f,151f
liver, 147
deficiency of, 152t
muscle, 147
absence of, 152t
activation of
calcium/muscle contraction and,
148
cAMP and, 147–148, 149f
Phosphorylase a, 147, 149f
Phosphorylase b, 147, 149f
Phosphorylase kinase
calcium/calmodulin-sensitive, in
glycogenolysis, 148
deficiency of, 152t
protein phosphatase-1 affecting, 147
Phosphorylase kinase a, 148, 149f
Phosphorylase kinase b, 148, 149f
Phosphorylation
in covalent modification, 76, 77–79, 78f,
78t
mass increases and, 27t
multisite, in glycogen metabolism, 151
oxidative. SeeOxidative phosphorylation
in respiratory burst, 623
Photolysis reaction, in vitamin D synthesis,
445
Photosensitivity, in porphyria, 274
Phototherapy, cancer, porphyrins in, 273
Phylloquinone, 482t,486, 488f.See also
Vitamin K
Physical map, 633, 634f
INDEX / 679
Physiologic (neonatal) jaundice, 282–283
Phytanic acid, Refsum’s disease caused by
accumulation of, 188
Phytase, 477
Phytic acid (inositol hexaphosphate), calcium
absorption affected by, 477
Pi, 589. See alsoα
1
-Antiproteinase
pI (isoelectric pH), amino acid net charge
and, 17
PI-3 kinase
in insulin signal transmission, 465, 466f
in Jak/STAT pathway, 467
PIC. SeePreinitiation complex
PIG-Agene, mutations of in paroxysmal
nocturnal hemoglobinuria, 531,
531f
“Ping-Pong” mechanism, in facilitated
diffusion, 427, 427f
Ping-pong reactions, 69–70, 69f
Pinocytosis, 429–430
PIP
2
, in absorptive pinocytosis, 430
Pituitary hormones, 437. See alsospecific
type
blood glucose affected by, 161
pK/pK
a
of amino acids, 15–16t,17, 17f,18
environment affecting, 18, 18t
medium affecting, 13
of weak acids, 10–11, 11–12, 12t,13, 17
PKA. SeeProtein kinase A
PKB. SeeProtein kinase B
PKC. SeeProtein kinase C
PKU. SeePhenylketonuria
Placenta, estriol synthesis by, 442
Plaque hybridization, 403. See also
Hybridization
Plasma, 580
Plasma cells, immunoglobulins synthesized
in, 591
Plasma enzymes. See alsoEnzymes
diagnostic significance of, 57, 57t
Plasma lipoproteins. SeeLipoproteins
Plasma membrane, 415, 426–431,426f.
See alsoMembranes
carbohydrates in, 110
mutations in, diseases caused by, 431,
432t
Plasma proteins, 514, 580–591,581f,583t.
See alsospecific type and
Glycoproteins
in bone, 548t
concentration of, 580
electrophoresis for analysis of, 580, 582f
functions of, 583, 583t
half life of, 582
in inflammation, 621t
polymorphism of, 582
synthesis of
in liver, 125, 581
on polyribosomes, 581
transport, 454–455,454t,455t,583t
Plasma thromboplastin antecedent
(PTA/factor XI), 599f,600, 600t
deficiency of, 601
Plasma thromboplastin component
(PTC/factor IX), 599f,600, 600t
coumarin drugs affecting, 604
deficiency of, 604
Plasmalogens, 116, 117f,199, 200f
Plasmids, 400–401, 401f,402, 402t,403f,
413
for cloning in gene isolation, 635t
Plasmin, 604–605, 604f
Plasminogen, 604
activators of, 604–605, 604f,605, 605f,
607t
Platelet-activating factor, 197, 621t
synthesis of, 198f,199, 200f
Platelets, activation/aggregation of, 598,
605–607, 606f
aspirin affecting, 607–608
Pleckstrin, in platelet activation, 607
PLP. SeePyridoxal phosphate
PNMT. SeePhenylethanolamine-
N-methyltransferase
pOH, in pH calculation, 9
Point mutations, 361
recombinant DNA technology in
detection of, 408–409, 408f,409t
Poisons, oxidative phosphorylation/
respiratory chain affected 
by, 92, 95,96f
Pol II
phosphorylation of, 350–351
in preinitiation complex formation,
351–352
in transcription, 350–351
Polarity
of DNA replication/synthesis, 330–331
of protein synthesis, 364
of xenobiotics, metabolism and, 626
Poly(A) tail, of mRNA, 309, 355–356
in initiation of protein synthesis, 365
Polyacrylamide gel electrophoresis, for
protein/peptide purification, 
24, 24f,25f
Polyadenylation sites, alternative, 394
Polyamines, synthesis of, 265–266, 266f
Polycistronic mRNA, 376
Polycythemia, 46
Polydystrophy, pseudo-Hurler, 532, 546t
Polyelectrolytes, peptides as, 19
Polyfunctional acids, nucleotides as, 290
Polyisoprenoids, in cholesterol synthesis,
220, 221f
Polyisoprenol, in N-glycosylation, 521–522
Polymerase chain reaction (PCR), 57,
405–406, 406f,413, 414
in gene isolation, 635t
in microsatellite repeat sequence
detection, 322
in primary structure determination, 26
Polymerases
DNA, 326, 327–328, 327f,328, 328t
in recombinant DNA technology, 400t
RNA, DNA-dependent, in RNA
synthesis, 342–343, 342f,343t
Polymorphisms, 407
acetyltransferase, 630
cytochrome P450, 628, 630t
microsatellite, 322, 411, 413
plasma protein, 582
restriction fragment length. See
Restriction fragment length
polymorphisms
single nucleotide, 414
Polynucleotide kinase, in recombinant
DNA technology, 400t
Polynucleotides, 291–292
posttranslational modification of, 289
Polyol (sorbitol) pathway, 172
Polypeptides
receptors for, 436
sequencing of
cleavage in, 25, 26t
Sanger’s determination of, 24–25
Polyphosphoinositide pathway, platelet
activation and, 605–607
Polyprenoids, 118, 119f
Polyribosomes (polysomes), 310, 370
protein synthesis on, 498, 499f,500f,
506
plasma proteins, 581
signal hypothesis of binding of,
503–505, 504t,505f
Polysaccharides, 102, 107–110,108f,109f.
See alsospecific type
Polysomes. SeePolyribosomes
Polytene chromosomes, 318, 318f
Polyunsaturated fatty acids, 112, 113t.See
alsoFatty acids; Unsaturated fatty
acids
dietary, cholesterol levels affected by, 227
eicosanoids formed from, 190, 192,
193f,194f
essential, 190,190f
synthesis of, 191,191f,192f
POMC. SeePro-opiomelanocortin
(POMC) peptide family
Pompe’s disease, 152t
Porcine stress syndrome, 565
Porphobilinogen, 270, 273f,275f
Porphyrias, 274–278,277f,277t
Porphyrinogens, 272
accumulation of in porphyria, 274–278
Porphyrins, 270–278,271f,272f
absorption spectra of, 273–274,277f
heme synthesis and, 270–273,273f,
274f,275f,276f
reduced, 272
spectrophotometry in detection of,
273–274
Positive nitrogen balance, 479
680 / INDEX
Positive regulators, of gene expression, 374,
375t,378, 380
Posttranslational processing, 30, 37–39,
38f,371
of collagen, 537–538, 537t
in membrane assembly, 511–512
Posttranslational translocation, 499
Potassium, 496t
in extracellular and intracellular fluid,
416, 416t
permeability coefficient of, 419f
Power stroke, 561
PPI. SeePeptidyl prolyl isomerase
PP
i
SeePyrophosphate, inorganic
PR. SeeProgesterone, receptors for
Pravastatin, 229
PRE. SeeProgestin response element
Pre-β-lipoproteins, 205, 206t,210
Precursor proteins, amyloid, 590
Pregnancy
estriol synthesis in, 442
fatty liver of, 188
hypoglycemia during, 161
iron needs during, 586
Pregnancy toxemia of ewes (twin lamb
disease)
fatty liver and, 212
ketosis in, 188
Pregnenolone, 440f
in adrenal steroidogenesis, 438–440,
440f,441f
in testicular steroidogenesis, 442, 443f
Preinitiation complex, 343, 351–352
assembly of, 351–352
in protein synthesis, 365, 366f
Prekallikrein, 599f,600
Premenstrual syndrome, vitamin B
6
in
management of, sensory
neuropathy and, 491
Prenatal diagnosis, recombinant DNA
technology in, 409
Preprocollagen, 537
Preprohormone, insulin synthesized as, 449,
450f
Preproparathyroid hormone (preproPTH),
450, 451f
Preproprotein, albumin synthesized as, 583
Preproteins, 498, 581
Presequence. SeeSignal peptide
Preventive medicine, biochemical research
affecting, 2
Primaquine-sensitive hemolytic anemia, 613
Primary structure, 21–29,31. See also
Protein sequencing
amino acid sequence determining, 18–19
Edman reaction in determination of, 25,
26f
genomics in analysis of, 28
molecular biology in determination of,
25–26
of polynucleotides, 291–292
proteomics and, 28–29
Sanger’s technique in determination of,
24–25
Primary transcript, 342
Primases, DNA, 327, 327f,328t
Primosome, 328, 414
Prion diseases (transmissible spongiform
encephalopathies), 37
Prion-related protein (PrP), 37
Prions, 37
Proaccelerin (factor V), 600t,601, 602f
Proaminopeptidase, 477
Probes, 402, 414. See alsoDNA probes
for gene isolation, 635t
Probucol, 229
Procarcinogens, 626
Processivity, DNA polymerase, 328
Prochymotrypsin, activation of, 77, 77f
Procollagen, 371, 496, 537
Procollagen aminoproteinase, 537
Procollagen carboxyproteinase, 537
Procollagen N-proteinase, disease caused by
deficiency of, 538t
Proconvertin (factor VII), 599f,600t,601
coumarin drugs affecting, 604
Prodrugs, 626
Proelastase, 477
Proenzymes, 76
rapid response to physiologic demand
and, 76
Profiling, protein-transcript, 412
Progesterone, 439f,440f
binding of, 455, 455t
receptors for, 471
synthesis of, 438, 442, 445f
Progesterone (∆4) pathway, 442, 443f
Progestin response element, 459t
Progestins, binding of, 455
Prohormones, 371
Proinsulin, 449, 450f
Prokaryotic gene expression. See alsoGene
expression
eukaryotic gene expression compared
with, 391–395,392t
as model for study, 375
unique features of, 375–376
Prolactin, 437
localization of gene for, 407t
receptor for, 436
Proline, 16t
accumulation of (hyperprolinemia),
249–250
catabolism of, 249–250, 251f
synthesis of, 238, 239f
Proline dehydrogenase, block of proline
catabolism at, 249–250
Proline hydroxylase, vitamin C as coenzyme
for, 496
Proline-cis,trans-isomerase, protein folding
and, 37, 37f
Prolyl hydroxylase reaction, 240, 240f,535
Promoter recognition specificity, 343
Promoters, in transcription, 342, 342f
alternative use of in regulation, 354–355,
355f,393–394
bacterial, 345–346, 345f
eukaryotic, 346–349, 347f,348f,349f,
384
Promotor site, in operon model, 377f,378
Proofreading, DNA polymerase, 328
Pro-opiomelanocortin (POMC) peptide
family, 452–453, 453f.See also
specific type
Pro-oxidants, 612. See alsoFree radicals
Proparathyroid hormone (proPTH), 450,
450f
Propionate
blood glucose and, 159
in gluconeogenesis, 154f,155
metabolism of, 155, 155f
Propionic acid, 112t
Propionyl-CoA
fatty acid oxidation yielding, 182
methionine in formation of, 259, 259f
Propionyl-CoA carboxylase, 155, 155f
Proproteins, 37–38, 76, 371
Propyl gallate, as antioxidant/food
preservative, 119
Prostacyclins, 112
clinical significance of, 196
clotting/thrombosis affected by, 607, 607t
Prostaglandin E
2
, 112, 113f
Prostaglandin H synthase, 192
Prostaglandins, 112, 113f,190, 192
cyclooxygenase pathway in synthesis of,
192, 192–194, 193f,194f
Prostanoids, 112, 119
clinical significance of, 196
cyclooxygenase pathway in synthesis of,
192, 192–194,193f,194f
Prosthetic groups, 50
in catalysis, 50–51,51f
Protamine, 603
Proteases/proteinases, 8, 477, 624t.See also
specific type
α
2
-macroglobulin binding of, 590
in cartilage, 553
as catalytically inactive proenzymes,
76–77
mucin resistance to, 520
of neutrophils, 623–624, 624t
in protein degradation, 242, 243f,477
Staphylococcus aureusV8, for polypeptide
cleavage, 25, 26t
Protein 4.1, in red cell membranes, 615f,
616f,616t,617
Protein C, in blood coagulation, 600t,603
Protein disulfide isomerase, protein folding
and, 37, 508
Protein-DNA interactions, bacteriophage
lambda as paradigm for,
378–383, 379f,380f,381f,382f
INDEX / 681
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