новая папка / 123

genetic testing for sister of, 320, 330 iron chelation for, 122

sickle cell disease in

genetic mutation in, 86, 88, 89, 90, 223, 278, 322, 330 hemoglobin S (HbS) in, 86, 88, 90, 94, 112, 121, 223, 884 protein quaternary structure in, 112

vaso-occlusive crisis in, 81, 90, 94, 121–122 steatorrhea in, 599

stroke prevention in, 121–122Subject Index

NOTE: Page numbers followed by f denote figures; page numbers followed by t denote tables.

AA

bbreviations, for amino acids, 83, 84f Abdominal fat, 30–31

Abetalipoproteinemia, 602–603, 604t, 648, 663t

ABO blood groups, 175, 544–545, 558–561, 558f, 560t Absorption, 3, 24, 25–26, 26f

amino acid, 24, 25, 738, 741–742 carbohydrate, 24, 25, 415, 425–428 cholesterol, 669

glucose, 415, 425–428, 578 iron, 875–876

lipid (fat), 25–26, 26f, 597–598 vitamin B12, 797–798, 797f Absorptive state. See Fed state Abzymes, 133

Acarbose, 419

Acceptable macronutrient distribution range (AMDR), 11 Acetaldehyde

adducts of, 702

in alcohol use disorder, 143, 184–185

ethanol oxidation to, 139, 139f, 143, 151, 154, 184, 702–707 metabolism to acetate, 702, 703, 705

toxicity of, 702, 710–711 tubulin inhibition by, 185

Acetaldehyde dehydrogenase (ALDH), 702, 703, 705 Acetaminophen, 645f

Acetaminophen detoxification, 916–917, 917f Acetate

acetaldehyde metabolism to, 702, 703, 705

acetyl coenzyme A from, 469, 702, 703–704, 705, 705f fate of, 705

as fuel for skeletal muscle, 947 Acetic acid, 51t, 52, 53f

Acetoacetate, 37, 37f, 63–65, 607, 621–623 alternative pathways of metabolism, 623

amino acid degradation to, 771, 771f, 780f, 781–783, 781f, 782f assays/identification of, 63, 64, 75oxidation of, 621–622 synthesis of, 621, 622f

Acetoacetate decarboxylase, 569 Acetoacetic acid, 51t, 55 Acetoacetyl-CoA thiolase, 611t

Acetoacetyl coenzyme A (acetoacetyl-CoA), 621, 666 Acetone, 37f, 63, 64, 622f

in diabetic ketoacidosis, 48, 63, 569, 570, 609 Acetylation, 93f, 94, 284

and fatty acid oxidation, 626–627, 627f histone, 294, 302, 302f, 626 Acetylcholine (ACh), 192–193, 965–966 inactivation and degradation of, 966, 966f in malathion poisoning, 140, 143

in muscle contraction, 935–936, 936f in myasthenia gravis, 191, 193, 206–207

as neurotransmitter, 192–193, 192f, 834, 953, 958, 965–966 paracrine signaling by, 193

synthesis of, 834, 953, 965–966, 966f in tardive dyskinesia, 966 Acetylcholine receptors

muscarinic, 193, 203

in myasthenia gravis, 193, 206–207 nicotinic, 192–193, 193f, 203

Acetylcholinesterase, 140, 143, 193, 206–207, 936, 966, 966f Acetylcholinesterase inhibition/inhibitors, 140, 141f, 193, 206–207, 936 Acetyl coenzyme A (acetyl-CoA), 4f, 5, 27, 37

acetate conversion to, 469, 702, 703–704, 705, 705f acetylcholine synthesis from, 965–966, 966f

amino acid conversion to, 375, 771, 771f, 781–783, 781f, 828, 830, 831f in cellular respiration, 372–373, 374f

in cholesterol synthesis, 666, 669–670, 670f

fatty acid oxidation to, 375, 469, 591, 609, 613–621 (See also Fatty acid oxidation) in fatty acid synthesis, 631, 634–637

in gluconeogenesis, 571–572, 576 high-energy bonds of, 403, 403f

ketone bodies from and to, 469, 591, 607, 608f, 621, 622f, 724–726 mevalonate synthesis from, 670, 670f

PDC kinase inhibition by, 470–471 precursors of, 469–471

pyruvate carboxylase activation by, 472 pyruvate oxidation to, 469–471

release in β-oxidation, 613–614, 613f sources of, 469, 470f

transport of, 472

in tricarboxylic acid cycle, 457, 457f, 458–459, 459f, 469–471, 571–572 Acetyl coenzyme A (acetyl-CoA) carboxylase, 721–723, 722f, 729t

AMPK and, 730

in fatty acid synthesis, 631, 636, 636f, 721–723 in heart (cardiac muscle), 939

in hepatic fibrosis, 714 in muscle, 932, 938, 938f

A-chain, of insulin, 382, 382f

Acid(s), 47, 50–52. See also specific acids blood, in healthy individual, 51t conjugate, 51

dissociation of, 51–52, 52finorganic, 47, 51 metabolic, 47, 51, 53–56

metabolic production of, 827, 828 organic, 51

strong, 47, 51–52, 51t undissociated, 47

urinary excretion of, 47, 55–56 weak, 47, 51–52, 51t

Acid anhydride, 64f

Acid–base catalysis, 128, 132, 133, 135 Acidic amino acids, 80, 84f, 85t, 86–88 Acidosis

alcohol-induced (ketoacidosis), 702, 703, 708, 709–710 diabetic (See Diabetic ketoacidosis)

la

ctic (See Lactic acidosis) Aconitase, 459, 467

Acquired immune deficiency syndrome. See HIV/AIDS Acromegaly, 849, 850, 851, 866t

Actin

erythrocyte, 879, 880f F-actin, 183, 184f

G-actin, 105–106, 106f, 183, 184f integrin binding to, 978

in muscle contraction, 932, 936, 937f Actin filaments, 169, 183–184, 184f Actin fold, 100, 106–107, 106f

Actin microfilaments, 169, 184

Action potential, 192–193, 192f, 935–936 Activated protein C complex (APC), 902, 903 Activation, 719, 729t. See also specific processes Activation energy, for enzymes, 128, 132, 132f Activation-transfer coenzymes, 134–136, 135f, 137f

Activators, 256, 294, 303–304, 304f. See also specific substances and processes allosteric, 150, 156, 157–158, 157f

Active site, of enzymes, 128, 129–130, 130f

Active sulfate. See 3′-Phosphoadenosine 5′-phosphosulfate Active transport, 169, 174, 175f

of amino acids, 738 carbohydrate transport via, 415 and cell death, 410, 410f

energy transformation for, 399–400 Na+-dependent, 415, 426

primary, 177 secondary, 177, 182

Active transport pumps, 174 Acute cholecystitis, 595, 602 Acute pancreatitis, 595, 597, 602 Acylcarnitine disorders, 612 Acyl carrier protein (ACP), 637

Acyl-CoA-cholesterol acyltransferase (ACAT), 674, 674f, 684 Acyl-CoA dehydrogenases, 611t, 614

Acyl-CoA synthetase, 607, 610–612, 610f, 611t, 617 Acylglycerols, 70–71

Acyltransferase, 144, 611t

Adaptation, gene expression regulation for, 295 Adaptive thermogenesis, 403Adducts, 702 Adenine, 73, 74f, 215t

degradation of, 817

in DNA, 213, 215–218, 215f, 217f pairing of, 216–218, 217f

in RNA, 223, 223f

Adenine nucleotide translocase (ANT), 481, 496, 497f, 498, 498f Adenine phosphoribosyltransferase (APRT), 806, 811, 812f Adenocarcinoma, 227t

colon, 214, 224, 225–226, 345, 346 lu

ng, 231, 345, 346 Adenoma, 214

Adenomatous polyposis coli (APC), 356, 356f, 361 Adenosine accumulation, in ADA deficiency, 819 Adenosine deaminase (ADA)

defect or deficiency, 336, 806, 819, 820t measuring activity of, 811

polyethylene glycol-modified, therapeutic use of, 819 in purine base salvage, 806, 811, 812f

Adenosine diphosphate (ADP), 4, 4f as allosteric activator, 158

ATP synthesis from, 394 (See also Adenosine triphosphate (ATP) generation) concentration relationship with ATP and AMP, 447–448, 448f

cycle with ATP, 4, 4f, 394–409

in deoxyribonucleotide production, 806, 817 PDC kinase inhibition by, 470

in platelet, 896

in platelet activation, 897 regulation of, 806

in tricarboxylic acid cycle, 458, 468–469

Adenosine diphosphate (ADP)-ribosylating factor, 182, 205 Adenosine diphosphate (ADP)-ribosylation, 93f, 94, 284 Adenosine kinase, 806, 811, 812f

Adenosine monophosphate (AMP)

as allosteric activator, 158, 435, 449

concentration relationship with ATP and ADP, 447–448, 448f degradation of, 817, 818f

in glycogen metabolism, 537–538, 537f, 726, 942–943, 943f glycogen phosphorylase activation by, 526, 942–943, 943f metabolic importance of, 726

in muscle metabolism, 942–943, 943f PFK-1 regulation by, 435, 449, 449f phosphorylation of, 810

protein kinase activated by (See AMP-activated protein kinase) in purine synthesis, 806, 808–811

synthesis of, 808–809, 809f

Adenosine monophosphate (AMP) deaminase, 817 Adenosine triphosphate (ATP)

actin fold binding of, 106–107, 106f in aminoacyl-tRNA formation, 279, 279f buffer function of, 55

cAMP synthesis from, 204–205, 205f chemical bond energy from, 394–403 for biochemical work, 394, 400–403

change in free energy during reaction, 395–398 for mechanical work, 394, 399, 399f

for transport work, 394, 399–400cleavage or hydrolysis of, 394, 396, 396f, 402–403 concentration relationship with ADP and AMP, 447–448, 448f

in deoxyribonucleotide production, 816–817 depletion, in hypoxia and cell death, 410, 410f

electron-transport chain for, 5, 369–375, 480–489, 480f energy available to do work, 395–398

as energy currency, 376, 394, 395 estimated daily use by tissue, 402 in glycogen metabolism, 537–538, 537f in insulin secretion, 382–383, 383f

for muscle contraction, 399, 399f, 936, 937f PFK-1 regulation by, 435, 449, 449f

in platelets, 896

in purine synthesis, 806, 807f, 808, 809 in pyrimidine synthesis, 813, 814f

as RNA precursor, 252

Adenosine triphosphate (ATP)-binding cassette (ABC) protein family, 176, 669, 681– 682

Adenosine triphosphate (ATP) generation, 4–5, 4f, 27–28, 375, 403–407. See also specific processes

in ATP–ADP cycle, 4, 4f, 394–409 binding-change mechanism in, 483–484, 484f in cellular respiration, 372–374, 374f, 394 chemiosmotic hypothesis of, 480, 480f, 482 electron-transport chain coupled to, 493–496

electron-transport chain uncoupling from, 481, 493, 494–496 chemical uncouplers in, 494, 495f

proton leak in, 481, 494, 496 uncoupling proteins in, 494–496, 495f energy required for, 394–395

in ethanol metabolism, 707–708

in fatty acid metabolism, 591, 607, 624, 946–947, 946f in glycolysis, 28, 369, 375, 434–454

anaerobic, 369, 370f, 403, 407, 407f, 434–435, 435f, 436 regulation by need for ATP, 446–450

in skeletal muscle, 939, 942–945

substrate-level phosphorylation in, 375, 434, 435f, 436, 438–440, 439f yield per mole of glucose, 434, 437, 489

metabolic homeostasis for, 377–380

in mitochondria, 169, 180, 374, 374f, 480–481

in oxidative phosphorylation, 5, 394–395, 403–407, 404f, 435, 480–500, 942 problems in, pathologic consequences of, 375

in red blood cells, 873, 873f in skeletal muscle, 939–948

from creatine phosphate, 939–941, 940f in glycolysis, 939, 942–945

in tricarboxylic acid cycle, 4f, 5, 27, 37, 375, 457 Adenosine triphosphate (ATP) homeostasis, 394, 403, 408 glycolysis and, 434–435, 447

oxidative phosphorylation and, 482 tricarboxylic acid cycle and, 467, 469 Adenosine triphosphate (ATP) synthase binding-change mechanism of, 483–484, 484f in cellular respiration, 373–374, 374f

in ETC–ATP coupling, 493–494, 494f in ETC–ATP uncoupling, 494–495

in oxidative phosphorylation, 480, 483–484 structure of, 483, 483f

Adenovirusesas cloning vectors, 327 as gene therapy vectors, 337 Adenylate cyclase, 203–205, 204f, 205f adrenergic receptors and, 388 eicosanoids and, 646

in energy-requiring reactions, 402–403 glucagon and, 386–387

in glycogen metabolism, 533, 534f, 536, 537f, 538 inhibition of, 387

insulin and, 389 isoforms of, 91, 91f

muscle (myokinase), 943, 943f posttranslational modification of, 92 Adenylate kinase reaction, 637, 726 Adenylosuccinate, 808–809, 809f Adenylosuccinate lyase, 809

Adenylosuccinate synthetase, 806, 810–811, 810f Adequate Intake (AI), 11, 13, 14t, 19

Adhesion

cell, 356, 356f platelet, 896–897, 896f

Adhesion proteins, 844, 978, 989–990 Adipocytes, 28

adiponectin secretion from, 657–658 development of, 660 differentiation of, 309

endocrine function of, 656–658 hyperplasia of, 30 hypertrophy of, 30

ketone body usage by, 624 le

ptin secretion from, 656–657 thyroid hormone and, 859 Adiponectin, 657–658, 660 Adipose tissue

brown, 494–496, 495f, 859–860 cells of (See Adipocytes) endocrine function of, 656–658 environmental toxins in, 75–76 fasting state

brief fast, 37 prolonged fasting, 38–40

fatty acid synthesis in, 634 fuel storage in, 3, 7–8, 24, 29

glucose metabolism in, 28, 580f, 582f glucose transport in, 428, 428f growth hormone and, 851

lipolysis in, 34, 36, 37, 608, 724 lipoprotein lipase production in, 601 metabolic capacities of, 38t metabolic specialization of, 1

triacylglycerol storage in, 632, 649, 650f, 723–724, 723f, 845 triacylglycerol synthesis in, 647–648, 647f

white, 494–495, 648

Adipose triglyceride lipase (ATGL), 649–651, 650f Adrenal androgens, 693, 693f. See also Androgen(s) Adrenal cortex

aldosterone synthesis in, 693androgen synthesis in, 693 cortisol synthesis in, 692–693, 692f

Adrenal corticotrophic hormone (ACTH), 197 Adrenal hyperplasia, 668

Adrenal neoplasms, 853, 954, 961 Adrenergic receptor(s), 388

α, 388, 536, 536f, 853–854

β, 107–108, 108f, 388, 536–537, 853–854 Adrenergic receptor blockers

α, 963, 973 β, 481, 963, 973

Adrenocorticotropic hormone (ACTH), 692f, 693, 694, 696–697, 855 in Cushing disease, 855, 856, 857, 864

ectopic syndrome, 864 Adrenoleukodystrophy, 181 Adriamycin (doxorubicin), 295, 482 Adult hypolactasia, 423, 423t

Adult isoform of hemoglobin, 80, 90, 108

Advanced glycosylation end products (AGEs), 119, 585, 991–992 Adventitia, 687, 688f

Advil (ibuprofen), 645, 645f

Affinity (association) constant, 101, 109 Aflatoxin B1, 916

A form of DNA, 218–220, 220f

Age-related macular degeneration (AMD), 518, 522t Aging

Cockayne syndrome and, 247, 248t lipofuscin and, 509

progeria and, 278 telomeres and, 240 AIDS. See HIV/AIDS

Akt (protein kinase B), 201, 201f, 360, 539, 730, 744f Alanine, 83, 84f, 85t

carbon sources for, 830, 831 codons for, 276, 276f

cycling with glucose, 586, 586f, 756–757, 756f, 832, 832f degradation of, 770–771, 771f, 776

to TCA cycle intermediates, 472, 472f fasting state, 761, 761f

for gluconeogenesis, 566, 571, 571f metabolism of, fasting state, 825–828, 826f nitrogen transport by, 751, 756–757, 756f, 823

synthesis of, 751, 756, 769, 770f, 772–773, 773f, 776 Alanine aminotransferase (ALT), 609, 712–713, 776

in gluconeogenesis, 571, 571f in hepatitis A, 755, 757

le

vels as sign of disease, 926–927 Albinism, 962, 975t

Albumin, 894, 918–919 deficiency of, 894 drugs binding to, 894

fatty acids bound to, 607, 608f, 610 in protein malnutrition, 38, 835, 894 steroid hormones bound to, 690 transport by, 196

Alcohol(s), 64, 64f

Alcohol, dietary. See also Ethanolcalories/energy yield from, 5t, 7, 407, 707–708 dietary guidelines on, 17–18

oxidation of, 7

Alcohol dehydrogenase (ADH), 702, 703–705

ethanol oxidation by, 139, 139f, 143, 151, 154, 184, 702, 703–705 isozymes of, 704–705, 704t, 706

metal-ion catalysis by, 139, 139f product inhibition of, 156 substrate concentration for, 154

Alcohol-induced cirrhosis, 702, 703, 712–714, 911, 918, 926–928 Alcohol-induced hepatitis, 710, 711f

Alcohol-induced ketoacidosis, 702, 703, 708, 709–710

Alcohol-induced liver disease, 702, 708–714, 911, 926–928. See also specific types Alcohol-induced megaloblastic anemia, 791, 792, 796, 803t

Alcoholism, 129, 151, 166t, 170, 187t, 715t ethanol oxidation in, 164, 184–185, 708 folate deficiency in, 791, 802 hypoglycemia in, 568, 584–585, 587t, 710 oxidative phosphorylation in, 711 pancreatitis in, 595, 597, 602, 604t

thiamin deficiency in, 129, 143, 147t, 458, 474 VLDLs in, 649

Aldehyde, 64, 64f

Aldehyde allysine, 980, 981f Aldohexose, 66, 66f

Aldolase(s), 145f, 146, 438, 439f, 440 Aldolase A, 441

Aldolase B, 435, 440, 441, 451 Aldolase C, 441

Aldose, 66

Aldose reductase, 435

Aldosterone, 57, 57f, 196f, 691f, 693 Aldosugars, 62, 66, 66f Alglucosidase alfa (Lumizyme), 530 Aliphatic amino acids, 80

nonpolar, 83–85, 85t polar, uncharged, 86

Aliphatic compounds, 63, 63f

Alkali, DNA/RNA effects of, 220, 220f Alkaline phosphatase, 755, 757, 865 Alkaptonuria, 781, 787t

Alkylating agents, 295 Alleles, 88, 222–223

polymorphisms in, 89 (See also Polymorphisms) Allele-specific oligonucleotide probes, 330 Allergic reactions, 638, 871, 965 Allopurinol, 140–142

for gout, 75, 129, 140–142, 143, 170, 185, 807, 818 mechanism of action, 140–142, 142f, 818, 818f Allosteric activators, 150, 156, 157–158, 157f Allosteric effectors, 156

Allosteric enzymes, 156–158

cooperativity in substrate binding to, 156–157, 157f in metabolic pathways, 158

Allosteric inhibitors, 150, 156, 157–158, 157f Allosteric site, 156, 157

All-trans retinoic acid, 196fAllysine, 118, 119f Alogliptin, 863

α-Adrenergic receptor(s), 388, 536, 536f, 853–854 α-Adrenergic receptor blockers, 963, 973 α-Amanitin, 252, 268

α-Amylase

pancreatic, 415, 418–419, 419f salivary, 25, 415, 418–419, 419f α2-Antiplasmin, 903

α1-Antiproteinase (AAP) deficiency, 895, 907t α1-Antitrypsin, 895

α1-Antitrypsin deficiency, 740 α-Carbon, 80, 81f, 82–83, 847

α-Cells of pancreas, 26, 376, 381, 384 α-Dextrins, 415, 419

α-Globin gene, 885–889, 886f α-Glucosidase, 530, 531t

α-1,4-Glycosidic bonds, 525, 525f, 529, 529f α-Granule, of platelets, 896

α-Helix, 100, 103, 103f 7α-Hydroxylase, 675–676, 675f, 676f 17α-Hydroxylase, 694 21α-Hydroxylase, 675–676, 675f, 676f α-Keto acid, 753, 753f, 781

α-Keto acid dehydrogenase complex, 464–466, 464f, 781, 787t α-Ketobutyrate, 774, 775f, 799

α-Ketoglutarate, 457, 457f, 459

amino acid degradation to, 771, 776–778

in amino acid nitrogen metabolism, 753–754, 753f, 754f amino acids related through, 776–778, 777f

amino synthesis from, 770f, 776–778 ammonia toxicity and, 760, 761–762 in cancer, 520–521

conversion to glutamate, GABA, and glutamine, 472, 760, 966–967 in gluconeogenesis, 571f

neurotransmitters from, 953, 966–967, 967f renal usage of, 828–829, 828f, 829f succinyl-CoA from, 460–461, 460f, 464, 464f

α-Ketoglutarate dehydrogenase complex, 457, 461, 464–465 compartmentation and, 474–475

in gluconeogenesis, 572 irreversible reactions of, 467 regulation of, 468f, 469 α-Lactalbumin, 554 α2-Macroglobulin, 895

α-Subunits of heterotrimeric G-proteins, 203–204, 204t α-Thalassemia, 252, 884

Alternative splicing, 310, 310f. See also Splicing reactions Altruistic organ, liver as, 844

AluI restriction enzyme, 321t Alu sequences, 266–267

Alzheimer disease, collagen in, 980 Amanita phalloides, 252, 268 Amenorrhea, anorexia nervosa and, 40 Amide(s), 64f. See also specific types

formation of, 65, 66fAmidophosphoribosyltransferase, 806, 810–811, 810f Amine(s)

biogenic, 194 (See also Neurotransmitters) charge of, 64, 65f

primary, 64, 65f quaternary, 64, 64f, 65f secondary, 64

tertiary, 64

Amino acid(s), 6, 80–97 abbreviations for, 83, 84f absorption of, 24, 25, 738, 741–742

acidic (negative), 80, 84f, 85t, 86–88 aliphatic, polar, uncharged, 86 α-carbon of, 80, 81f, 82–83

aromatic, 80, 84f, 85–86, 85t

basic (positive), 80, 84f, 85t, 86–88 biosynthesis from, 29 branched-chain, 83, 84f

charged, 80, 84f, 85t

chemical properties of, 80, 82–83

codons for, 275–277, 276t (See also Codons)

conditionally essential, 12 D-configuration of, 82, 82f degradation of, 770–771, 771f, 823

acetyl-CoA from, 375, 771, 771f, 781–783, 781f α-ketoglutarate from, 771, 776–778

carbon dioxide from, 770

fumarate from, 771, 771f, 776, 778

ketone bodies from, 623, 770–771, 771f, 780f, 781–783, 781f oxaloacetate from, 776, 778

pyruvate from, 770–771, 771f

succinyl-CoA from, 771, 771f, 774, 775f, 776, 779–781, 779f, 780f TCA cycle intermediates from, 472, 472f, 771, 771f, 776–781 digestion of, 24, 25, 26f, 738–741

electrophoresis identifying, 88, 90 essential, 3, 11, 12, 769

fasting state, 36, 37, 38, 752f, 825–828, 826f fed state, 24, 24f, 29, 752f

free, 12, 29, 769, 823–828

interorgan flux in postabsorptive state, 825–828, 826f maintenance of pool in blood, 824–828, 825f

membrane attack/lipid peroxidation by, 509–510, 509f, 510f source of, 823, 824, 825f

functional groups of, 80, 81f, 134, 134t general properties of, 80

glucagon regulation by, 384

glucogenic, 34, 566, 570, 575, 770, 771f, 828 glucose cycling with, 586, 586f, 773

insulin regulation by, 384

ketogenic, 623, 770–771, 771f, 780f, 781–783, 781f la

rge neutral, 957–958 L-configuration of, 80, 82, 82f

modified, 81, 91–94, 93f, 274, 284–285 neurotransmitter, 834

nonessential, 769, 770f nonpolar aliphatic, 83–85, 85t

oxidation of, 4–5, 4f, 375peptide bonds of, 80, 82–83, 83f posttranslational modifications of, 81

properties of, 85t

protein synthesis from, 274–290 (See also Protein synthesis) replenishing pool of (protein turnover), 29, 37, 119, 738, 743–746 side chains of, 80, 81f, 82–83

classification of, 80, 83–88, 84f hydrophobicity of, 80, 83, 84f polarity of, 80, 83, 84f

small neutral, 958

structure of, 6, 6f, 62, 72–73, 73f, 80, 81f, 82–83 substitutions in primary structure, 80–81, 88–91 sulfur-containing, 84f, 85t, 86, 86f

synthesis of (See Amino acid synthesis) transport of, 738

active, 738

blood–brain barrier and, 957–958 into cells, 742

defects in, 742, 743, 746, 748t

facilitative transporters for, 738, 741, 741f, 742 sodium-dependent transporters for, 738, 741–742, 741f systems of, 742t

uncharged polar, 80, 84f, 85t zwitterions, 80, 81f, 82

Amino acid metabolism, 29, 751, 752f, 769–786, 770–771, 771f acetyl-CoA from, 375, 771, 771f, 781–783, 781f, 830, 831f α-ketoglutarate from, 771, 776–778

brain and nervous tissue, 755, 827, 834, 834f, 835f carbon dioxide from, 770

carbon fate in, 751, 751f, 770–771 catabolic state, 823, 827–828

changes with dietary and physiologic state, 835–836 cofactors in, 769, 772

disorders of, 772, 784–785, 786t, 787t fasting state, 36, 37, 38, 752f, 825–828, 826f hepatic, 826–827, 826f

muscle, 36, 37, 825–827, 826f fed state, 24, 24f, 29, 752f

fumarate from, 771, 771f, 776, 778 glycolysis and, 769, 770–771, 772–776 gut, 832–833, 833f, 835, 835f

hepatic, 29, 751, 752f, 770, 823, 833–834, 919, 925–926 fasting state, 826–827, 826f

after high-protein meal, 835–836, 835f in liver disease, 926

homeostasis role of, 378–379 hypercatabolic state, 823, 836 intertissue relationships in, 823–839

ketone bodies from, 623, 770–771, 771f, 780f, 781–783, 781f muscle, 36, 37, 751, 752f, 755–757, 823, 830–832, 947 conversion of amino acids to glutamine, 830–832, 831f fasting state, 825–827, 826f

after high-protein meal, 835–836, 835f in hypercatabolic states, 836

oxidation of branched-chain amino acids, 823, 825, 827, 830, 831f, 941, 947 neurotransmitters from, 834, 844

nitrogen fate in, 751, 753–757conversion to urea, 34, 36, 37, 38, 751, 757–765 disorders of, 761–764

fasting state, 760–761, 761f, 825–828

in glucose–alanine cycle, 756–757, 756f, 832, 832f glutamate and, 753–756, 753f, 754f, 755f, 756f

in kidney, 751, 752f, 828–829, 828f removal as ammonia, 34, 753–755, 754f transamination reactions, 753, 753f transport to liver, 751, 756–757, 756f, 823 oxaloacetate from, 776, 778

principles governing interorgan flux, 827–828 pyruvate from, 770–771, 771f

renal, 751, 752f, 828–829, 828f, 829f

in sepsis and trauma, 823, 823f, 836, 838f

succinyl-CoA from, 771, 771f, 774, 775f, 776, 779–781, 779f, 780f, 830, 831f TCA cycle and, 469, 472, 472f, 769, 771, 771f, 776–781, 830, 831f

Amino acid pool, 823–828

interorgan flux in postabsorptive state, 825–828, 826f maintenance in blood, 824–828, 825f

principles governing interorgan flux, 827–828 source of, 823, 824, 825f

Amino acid sequence. See Primary structure of proteins Amino acid synthesis, 769, 772–778

α-ketoglutarate in, 770f, 776–778

glucose conversion in, 371, 371f, 375, 769, 770–771, 772–776 glutamate and, 755, 755f, 776–778, 777f

glycolytic pathway of, 769, 770–771, 772–776, 773f oxaloacetate in, 770f, 776, 778, 778f

regulation of, 769

TCA cycle intermediates and, 471–472, 471f, 769, 776–781 Aminoacyl (A) site, on ribosome, 280, 280f, 282–283, 282f Aminoacyl-transfer RNA, 274, 276, 278–284

binding to A site, 282–283, 282f formation of, 278–279, 279f Aminoacyl-tRNA synthetases, 278–279, 279f Amino groups, 62, 64f, 80, 81f, 82–83 Aminolevulinic acid (ALA), 874–875, 875f Aminopeptidases, 738