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.pdfin glycolysis, 369, 434–435, 434f, 437–438, 437f, 439f, 725f hexokinase inhibited by, 434, 448–449
metabolism of, 152, 153, 369–371, 437, 437f
in pentose phosphate pathway, 370f, 371, 371f, 437, 543–550, 543f, 545f polarity and solubility of, 65, 66f
product inhibition by, 155 in red blood cells, 874 supply of, regulation of, 447
Glucose 6-phosphate dehydrogenase, 549–550, 635, 721, 722f, 723, 729t, 874, 921 Glucose 6-phosphate dehydrogenase deficiency, 544–545, 549, 559–560, 563t, 890t Glucose–alanine cycle, 586, 586f, 756–757, 756f, 832, 832f
Glucose-dependent tissues, 34
Glucose homeostasis, 372, 376, 376f, 437
Glucose metabolism, 4–5, 27–28, 369–375. See also specific pathways and processes in adipose tissue, 28, 580f, 582f
ATP generation in, 4–5, 27–28, 369–375, 375 (See also Adenosine triphosphate (ATP) generation)
in basal (postabsorptive) state, 35, 36f
in brain and neural tissues, 27–28, 34, 444, 566, 579, 582f, 953, 968
in fasting, prolonged (starvation), 38, 39, 568f, 578, 579t, 583, 583f, 584, 584f in fasting state, 34, 35–40, 39f, 437, 526, 566, 568f, 578, 581–582, 584, 584f, 724– 726
in fed state, 24, 24f, 27–28, 437, 566, 568f, 579–581, 584, 584f, 720–723 GLP-1 and GIP in, 860–863, 862f, 862t
in glycolysis, 27, 28, 369–371, 375, 375f, 434–454, 922 anaerobic, 370f, 379, 403, 407, 407f, 434–435, 435f, 436 rate-limiting enzyme of, 434–435, 434f
substrate-level phosphorylation in, 434, 435f, 436 homeostasis in, 372, 376, 376f
intermediates of, 369, 370f in kidney, 569, 582f
in liver, 27, 34–38, 568–569, 579, 582f, 917–918, 921–922 major pathways of, 369f, 373f
in metabolic syndrome, 660–662
in muscle, 28, 28f, 580, 580f, 582f, 726, 942–945 AMP activation in, 942–943, 943f
in glycolysis/anaerobic glycolysis, 444, 938, 939, 942–945 in type IIb fast-twitch fibers, 942
neonatal, 526
in pentose phosphate pathway, 369, 370f, 371, 543–550, 543f
in red blood cells, 28, 34, 36, 575, 578–580, 582f, 843, 872–874, 873fin glycolysis, 444–445, 843, 869, 873–874, 873f
in pentose phosphate pathway, 548–549, 549f, 843, 869, 873f, 874 in tricarboxylic acid cycle, 369, 370f, 375
in various tissues, 38t, 579–580, 580f Glucose monitor, blood, 75
Glucose sparing, 38
Glucose tolerance, impaired, 56 “Glucose toxicity,” 991
Glucose transporters, 176, 382–383, 415, 425–428 blood–brain barrier and, 428, 429f, 957 cardiac, 939
cholera and, 430
deficiency of (GLUT 1), 957, 975t facilitative, 415, 426, 427f, 427t fructose, 440
galactose and fructose absorption through, 426–427 Na+-dependent, 415, 426, 427f, 430
tissue specificity of, 428, 428f Glucosidase, 530, 531t Glucuronate, 551–553, 551f, 552f Glucuronic acid, 68, 69f, 985f, 986
Glucuronides, 544, 551–553, 552f, 553t, 879 GLUT 1 transporter, 426, 427t, 428, 939, 957 GLUT 1 transporter deficiency, 957, 975t
GLUT 2 transporter, 382, 426, 427t, 428 GLUT 3 transporter, 426, 427t, 428, 957
GLUT 4 transporter, 426, 427t, 428, 428f, 726, 939 GLUT 5 transporter, 426, 427t, 440
Glutamate, 84f, 85t, 86, 966–967 α-ketoglutarate conversion to, 472, 760, 966–967
in amino acid nitrogen metabolism, 753–756, 753f, 754f, 755f, 756f amino acids related through, 755, 755f, 776–778, 777f
ammonia toxicity and, 762 deamination of, 754–755, 754f degradation of, 771, 771f, 776 excitotoxicity of, 953, 969 functions of, 769
GABA synthesis from, 967, 967f histidine conversion to, 771
as neurotransmitter, 834, 953, 958, 966–967 in sickle cell hemoglobin, 86, 87, 88 storage and release of, 967
synthesis of, 769, 770f, 776, 966–967, 967f TCA cycle intermediates and, 472, 970 transamination to, 753, 753f
transport of, 967
in urea cycle, 755–756, 756f
Glutamate dehydrogenase, 751, 754–756, 754f, 776, 966, 967f Glutamate-oxaloacetate transaminase, 145f
Glutamate semialdehyde, 759, 759f, 777–778, 777f Glutaminase, 751, 755, 757, 757f, 762, 776, 777f Glutamine, 84f, 85t, 86
α-ketoglutarate conversion to, 472, 760 ammonia toxicity and, 762
deamination of, 755
degradation of, 771, 771f, 776, 777ffunctions of, 823, 827, 828t glutamate synthesis from, 967, 967f
glutamate transport in, 967 metabolism of
brain, 834, 834f
fasting state, 825–828, 826f hypercatabolic states, 836 muscle, 823, 827, 830–832 renal, 828–829, 828f, 829f
in sepsis and trauma, 823, 823f, 836 nitrogen transport by, 751, 756–757, 823 oxidation of, 751
in purine synthesis, 806, 806f, 807f, 808–811, 808f in pyrimidine synthesis, 813, 814f
synthesis of, 751, 757, 757f, 770f, 776, 777f and TCA cycle, 472
Glutamine phosphoribosylamidotransferase, 808, 808f, 810–811, 810f Glutamine synthase, 751, 755, 757, 757f
Glutamine synthetase, 776, 777f Glutathione
defense system, 505, 515–516, 516f, 543, 548–549, 549f hepatic usage of, 920–921
synthesis of, 746–747, 747f, 776, 919t Glutathione peroxidase, 505, 515–516, 516f Glutathione reductase, 515–516, 516f, 920–921 Glycation. See Glycosylation
Glycemic index, 425–426, 425t Glyceraldehyde, D- and L-forms of, 66–67, 67f
Glyceraldehyde 3-phosphate, 437, 438, 439f, 440–441, 440f, 543f, 544, 725f generation of ribose 5-phosphate from, 548, 548f
ribulose 5-phosphate conversion to, 546–547, 547f Glyceraldehyde-3-phosphate dehydrogenase, 438, 443, 452–453, 453f Glycerol, 62
cycling with glucose, 586, 586f
fasting state, 34, 36, 37, 582
for gluconeogenesis, 566, 570, 571, 571f, 575
as triacylglycerol backbone, 6, 6f, 70–71, 71f, 594, 595, 595f triacylglycerol release of, 632, 632f, 649–651, 650f
Glycerol 3-phosphate
in fatty acid storage, 649, 650f in gluconeogenesis, 568, 571f in glyceroneogenesis, 651
in glycerophospholipid synthesis, 632, 651–652 shuttle, in glycolysis, 434, 434f, 488, 488f in triacylglycerol synthesis, 632, 647f, 648
Glycerol 3-phosphate acyltransferase (GPAT), 730 Glycerolipids, 651–656. See also Glycerophospholipids Glycerol kinase, 647f, 648
Glyceroneogenesis, 650–651 Glycerophospholipids, 591, 651–656 degradation of, 654–656, 655f ether, 632, 651, 652–654, 654f functions of, 651
interconversion among, 652, 653f metabolism of, 632, 651–656 structure of, 632, 633f
synthesis of, 632, 651–652, 652f, 653ftypes of, 651f Glycine, 83, 84f, 85t, 968
in bile salt conjugation, 676, 677f in collagen, 979, 993
in creatine synthesis, 940f, 941 degradation of, 770–771, 771f, 774, 774f disorders of, 786t
functions of, 769
in heme synthesis, 843, 869, 874–875, 875f as neurotransmitter, 834, 953, 958, 968
as one-carbon group recipient, 794, 795f, 795t
as one-carbon group source, 790, 790f, 794, 794f, 795t in osteogenesis imperfecta, 993
in purine synthesis, 806, 806f, 807f, 808, 809f synthesis of, 769, 770f, 772, 773f, 774, 774f, 953, 968 Glycine transaminase, 786t, 787t
Glycocalyx, 171f, 172, 174 Glycochenodeoxycholic acid, 919t Glycocholic acid, 676, 677f, 919t Glycogen, 3, 5, 5f, 7–8, 7t, 525–540, 719 anorexia nervosa and, 152
degradation of, 525–530, 528f, 530f (See also Glycogenolysis) as emergency fuel in glycolysis, 436–437, 527
fasting state, 34–38, 532–533, 532t, 533t, 566, 581, 724–726 fed state, 24, 24f, 532, 532t, 566, 720–723
function of, 527–528
glycosidic bonds of, 525, 525f, 529, 529f
hepatic metabolism of, 34–38, 526, 527, 531–537, 532t, 534f, 566, 720–723 blood glucose levels and, 535
calcium and, 535–536, 536f diabetes mellitus and, 535
epinephrine and, 534f, 535–537, 536f, 853, 854f
fasting state, 34–38, 532–533, 532t, 533t, 566, 581, 724–726 fed state, 720–723
glucagon in, 526, 531, 533–535, 534f, 845, 848 insulin in, 526, 531, 533, 535, 845, 847 mechanisms affecting, 720–723
nomenclature for enzymes in, 533 phosphorylation in, 533–534, 534f protein phosphatases in, 535
hepatic stores of, 7, 7t, 27, 371, 525, 527, 527f, 720 metabolic capacities of various tissues, 38t metabolic disorders of, 531, 531t
muscle metabolism of, 525–527, 527f, 532, 532t, 537–538, 537f, 726, 942–943 AMP activation of, 942–943, 943f
ATP availability and, 537 calcium and, 526, 532, 538 diabetes mellitus and, 535
epinephrine and, 526, 532, 853, 854f, 943, 944f muscle stores of, 7, 7t, 371, 525–526, 527, 527f, 943 neonatal stores of, 526, 536, 538–539
nonreducing ends of, 527, 529, 529f polymers and particles of, 527 structure of, 525, 525f, 527 synthesis of (See Glycogen synthesis) Glycogenin, 529
Glycogenolysis, 34–38, 152, 372, 372f, 376, 525–538, 528f, 530fdebranching enzyme in, 525, 528, 528f, 529–530, 530f
disorders of, 531, 531t epinephrine in, 526
gluconeogenesis activation with, 527 glucose 6-phosphate in, 437, 525, 527
in liver, 526, 527, 527f, 531–537, 532t, 534f blood glucose levels and, 535, 917–918 calcium and, 535–536, 536f
diabetes mellitus and, 535 epinephrine and, 534f, 535–537, 536f
fasting state, 34–38, 532–533, 532t, 533t, 566, 581, 724–726 glucagon and, 526, 531, 533–535, 534f, 845, 848
insulin and, 526, 531, 533, 535, 845, 847 nomenclature for enzymes in, 533 phosphorylation and, 533–534, 534f protein phosphatases and, 535
regulation of, 531–537, 532t
in muscle, 525–527, 527, 527f, 532, 532t, 537–538, 537f, 942–943 AMP activation of, 942–943, 943f
ATP availability and, 537 calcium and, 526, 532, 538 diabetes mellitus and, 535
epinephrine and, 526, 532, 943, 944f regulation of, 532, 532t, 537–538, 726 regulation of, 531–538
Glycogen phosphorylase, 525–526, 528f, 529, 530f, 531t, 729t covalent modification of, 533
disorders of, 530, 531, 531t in liver, 533–535, 534f
in muscle, 158–159, 159f, 160, 526, 538, 942–943, 943f protein phosphatases and, 535
Glycogen phosphorylase a, 533 Glycogen phosphorylase b, 533 Glycogen phosphorylase kinase, 159
Glycogen storage diseases, 531, 531t, 540t type II, 530
type III, 533, 534 type V, 531, 531t, 532 type VI, 531, 531t, 532
von Gierke disease, 531t, 535
Glycogen synthase, 144, 525, 528f, 529, 529f, 531t, 533–535, 721, 943 covalent modification of, 533
diabetes mellitus and, 535 inhibition of, 533–534, 534f, 539 muscle, 538
protein phosphatases and, 535 regulation of, 721, 721f, 729t Glycogen synthase a, 533 Glycogen synthase b, 533, 535
Glycogen synthase kinase-3 (GSK-3), 539
Glycogen synthesis, 27, 371, 372f, 525–538, 528f, 529f
activated intermediates in, 400f, 401 branching enzyme in, 525, 528f, 529 energetics of, 400–403, 400f
Gibbs free energy change in, 400–401 glucose 6-phosphate in, 437, 525, 528, 528f
in liver, 34–38, 526, 527, 527f, 531–537, 532t, 534fblood glucose levels and, 535, 917–918
calcium and, 535–536, 536f diabetes mellitus and, 535
epinephrine and, 534f, 535–537, 536f
fasting state, 34–38, 532–533, 532t, 533t, 566, 581, 724–726 fed state, 720–723
glucagon in, 526, 531, 533–535, 534f, 845, 848 insulin in, 526, 531, 533, 535, 845, 847 mechanisms affecting, 720–723
nomenclature for enzymes in, 533 phosphorylation in, 533–534, 534f protein phosphatases in, 535
in muscle, 525–527, 527f, 532, 532t, 537–538, 537f ATP availability and, 537
calcium and, 526, 532, 538 diabetes mellitus and, 535 epinephrine and, 526, 532
phosphoglucomutase reaction in, 398, 398f, 402, 528, 528f phosphoryl transfer reactions in, 400–401, 401t regulation of, 527–528, 531–538
insulin/glucagon ratio in, 526, 531 in liver, 531–537, 532t
in muscle, 532, 532t, 537–538
UDP-glucose in, 371, 400f, 401, 525, 528–529, 528f, 529f Glycochenodeoxycholic acid, 676
Glycolipids, 62, 171f, 172, 558–559, 651f as cell recognition factors, 544 digestion of, 415
function of, 558
structure of, 558, 558f, 633f synthesis of, 371–372, 372f, 544, 633 sugar precursors for, 554, 554t, 556f
Glycolysis, 27, 28, 369–371, 375f, 434–454, 922 as anabolic pathway, 437
anaerobic (See Anaerobic glycolysis) anorexia nervosa and, 152
ATP generated in, 28, 369, 375, 407, 407f, 434, 434f, 489 ATP-generating phase of, 437, 437f
biosynthetic function of, 437, 446, 446f capacities of various tissues, 38t
for fatty acid synthesis, 635, 635f
functions other than ATP generation, 435, 446, 446f gluconeogenesis as reversal of, 566, 567f, 569–570, 572 glucose 6-P conversion to triose phosphates in, 438, 439f glucose conversion to glucose 6-P in, 437–438, 437f, 439f glycogen as emergency fuel in, 436–437, 527
hexokinase initiation of, 434, 434f high-energy bonds in, 438–440, 452–453 intermediates of
amino acids from, 769, 770–771, 772–776, 773f fructose conversion to, 435, 435f, 436, 440–441, 440f galactose conversion to, 435, 436, 442, 443f generation of ribose 5-phosphate from, 548, 548f
ribulose 5-phosphate conversion to, 546–547, 546f, 547f muscle, 449, 938
in cardiac muscle, 445, 450, 938, 939
in skeletal muscle, 444, 938, 939, 942–945overview of, 434, 434f oxidation in, 438–440, 439f
oxidative fates of pyruvate and NADH in, 443–444, 443f
pentose phosphate pathway and, 543–550, 543f preparative phase of, 437–438, 437f, 439f
pyruvate generated for fatty acid synthesis in, 435, 437, 446 rate-limiting enzyme in (PFK-1), 434–435, 434f, 438, 439f, 447, 449–450 reactions of, 437–440
in red blood cells, 843, 869, 873–874, 873f regulation of, 446–450, 724–726, 725f enzyme activity/amount in, 575, 576f, 577t isoenzymes in, 447–449
major sites of, 447, 448f
regulation of hexokinases, 448–449, 448f regulation of PFK-1, 448f, 449–450, 449f regulation of pyruvate kinase, 450
relationships among ATP, ADP, and AMP concentrations, 447, 448f shuttle systems in, 434, 434f, 443–444
substrate-level phosphorylation in, 375, 434, 435f, 436, 438–440, 439f summary of pathway, 440
TCA cycle link to, 457, 469, 471 Glycopeptide transpeptidase, 141f Glycophorin, 173f
Glycophosphatidylinositol (GPI) glycan anchor, 174, 174f Glycoprotein(s), 62, 69, 171f, 172, 556–558 distribution in body, 544
function of, 556
in hepatic fibrosis, 927–928
plasma, 894 (See also Plasma proteins) structure of, 556, 556f
synthesis of, 371–372, 372f, 544, 556–558, 919–920 dolichol phosphate in, 556–558, 557f
sugar precursors for, 554, 554t, 556f Glycoprotein Ia, 897
Glycoprotein Ib, 897 Glycoprotein IIb-IIIa, 897
Glycosaminoglycans, 69, 551f, 844, 978, 984–988 functions of, 986, 986t
repeating disaccharides of, 984–986, 985f types of, 986
Glycosidases, 179, 418, 419–421, 420t Glycosides, 69
Glycosidic bonds, 62, 69, 70f, 415, 416f α-1,4, in glycogen, 525, 525f, 529, 529f hydrolysis of, 415, 418
in interconversions of sugars, 550 Glycosphingolipids, 72, 172
Glycosylated hemoglobin (A1C), 27, 56, 101, 120, 122, 379, 570, 863 Glycosylated proteins, 119, 120f
Glycosylation, 92, 285, 585, 687 nonenzymatic, 119, 120f
Glycosyltransferases, 144, 544, 550–551, 551f, 986–987, 987f Glyoxylate, 774, 774f
Goiter, 859, 860 diffuse toxic, 481
Golgi complex, 169, 170f, 182
collagen synthesis in, 982–983, 983tHMG-CoA reductase regulation in, 673, 674f la
minin synthesis in, 984
protein targeting from, 274, 286–287, 286f proteoglycan synthesis in, 978
in receptor-mediated endocytosis, 179–180 sphingolipid production in, 559
VLDL processing in, 648, 650f
Gout, 63, 74, 75, 129, 143, 147t, 170, 185, 187t, 807, 818, 820t allopurinol for, 75, 129, 140–142, 142, 142f, 143, 170, 185, 807, 818 colchicine for, 75, 143, 170, 183, 185, 807
inflammatory response in, 74, 180, 185, 195
le
ukotrienes in, 195
purine nucleotides in, 817, 818
uric acid/urate crystals in, 74, 180, 818 gp41 protein, 269, 270f
gp120 protein, 269, 270f G-protein(s)
adrenergic receptors and, 388 heterotrimeric, 107, 190, 203–204, 204f, 204t monomeric, 150, 160–161, 161f
as oncogenes, 349–350, 350t Ras superfamily of, 161
G-protein–coupled receptors (GPCRs), 198, 198f Gram-negative bacteria, 214
Gram-positive bacteria, 214 Gram stain, 214 Granulocytes, 871
Granulomatous disease, chronic, 513, 522t Grapefruit juice, as CYP3A4 inhibitor, 915 Graves disease, 481, 499, 500t
Grb2 protein, 199, 199f
Group transfer reactions, 144, 145f Growth factor(s), 190, 195, 308 hematopoietic, 881–883 hematopoietic, recombinant, 334
in hepatic fibrosis, 928 oncogenic, 349, 350t, 351f
Growth-factor receptors, oncogenic, 349, 350t, 351f Growth hormone, 843, 846t, 849–853 counterregulation by, 843, 845, 846t
diabetogenic potential of, 847, 851, 864 effects of
on adipose tissue, 851 direct, 850
on energy metabolism, 851, 852f indirect, 850
on liver, 851–853 on muscle, 851 human
recombinant production of, 333 structure of, 849, 849f receptors of, 850
secretion of, regulation of, 850, 851f, 852f somatostatin and, 849, 850
tumors secreting
in acromegaly, 847, 849, 850, 851
diagnosis of, 864Growth hormone release-inhibiting hormone (GHRIH), 850, 851f, 852f Growth hormone-releasing hormone (GHRH), 850, 851f, 852f
Growth-suppressor genes, 212 GTPase-activating proteins (GAPs), 206, 355 Guanase, 817
Guanidinoacetate, 940f, 941 Guanine, 74f, 215t degradation of, 817
in DNA, 213, 215–218, 215f, 215t, 217f pairing of, 216–218, 217f
Guanine nucleotide exchange factor (GEF), 199, 199f Guanosine, in mRNA, 251
Guanosine diphosphate (GDP)
in deoxyribonucleotide synthesis, 806 regulation of, 806
Guanosine monophosphate (GMP) degradation of, 817, 818f phosphorylation of, 810
in purine synthesis, 806, 807f, 809–811
synthesis of, 809, 810f Guanosine triphosphate (GTP)
in activated intermediate formation, 402 in AMP synthesis, 808–809, 809f proteins binding. See G-protein(s)
in protein synthesis, 274, 279–284 in purine synthesis, 807f, 808–809 as RNA precursor, 252
in tricarboxylic acid cycle, 457, 457f, 459, 460f, 461 tubulin binding of, 183
Guanylate cyclase, 968, 968f Guanylyl cyclase receptors, 207 Guide strand, of microRNAs, 312–313 Gums (dietary fiber), 424, 424t Gut (intestinal epithelium)
amino acid absorption in, 738
amino acid metabolism in, 751, 752f, 753, 754f, 832–833, 833f, 835, 835f cholesterol absorption in, 669
cholesterol metabolism in, 674–675 fiber metabolism in, 424
glucose metabolism in, 27
protein digestion in, 738, 739–741 sugar absorption in, 426–427 vitamin B12 absorption in, 797 xenobiotic biotransformation in, 914
Guthrie bacterial inhibition assay, 772 Gynecoid pattern, of fat storage, 30 HHA
ART, 246
Haber-Weiss reaction, 504, 506, 507f, 515 HaeIII restriction enzyme, 321t
Hairpin loop, 104, 104f Haploid cells, 221, 222f, 265 Haptocorrins, 797 Haptoglobin, 895t
Hartnup disease, 742, 743, 746, 748t
HDL. See High-density lipoproteinHealthy body weight, 10 Heart, 932, 934f, 935
bioenergetics of, 395, 402 fuel use in, 938–939 fatty acid, 624, 939
in ischemic conditions, 939 la
ctate, 939
in normal conditions, 939 glycolysis in, 445, 450, 938, 939 SERCA pump in, 948–949
Heart attack. See Myocardial infarction Heart failure
alcoholism and, 129, 143, 147t, 458, 474, 476t congestive, 409, 458, 476t
doxorubicin and, 482 le
ft ventricular, 395, 409
Heat, energy transformation to, 394, 403 Heat of fusion, water, 49
Heat production (thermogenesis), 403 adaptive, 403
definition of, 403 diet-induced, 8, 10 nonshivering, 494–496, 495f shivering, 403
thyroid hormone and, 859–860 Heat-shock proteins
in mitochondrial import, 180–181, 475, 475f
in protein folding, 117, 117f receptors associated with, 196–197 in steroid hormone binding, 305f, 306
Heavy (H) chains of immunoglobulins, 114–116, 115f Heavy-metal toxicity, 142–143
Hedgehog receptor, 350t, 355, 356f Height, 30
Height table, 12
Helicases, 230, 232, 232f, 238t Helix–loop–helix–leucine zipper, 673 Helix–loop–helix motif, 307f, 308 Helix–turn–helix motif, 307f, 308 Hematocrit, 436
Hematologic system, 843–844, 870. See also specific components Hematoma, 894, 904
Hematopoiesis, 843, 869, 880–883 cytokines and, 880–882
differentiation and hierarchy in, 880, 881f progenitor cells in, 880–881, 881f Hematopoietic growth factors, 881–883 Hematopoietic growth factors, recombinant, 334
Hematopoietic stem cells, 843, 869, 880–883, 881f Hematopoietic stem cell transplantation, 362 Heme, 29, 874–879
in cytochromes, 485, 486f, 874 degradation of, 81, 598, 877–879, 878f disorders of, 843, 869, 875f, 876
and eukaryotic initiation factors, 281, 311, 311f iron oxidation state in, 486, 487
and oxygen binding, 110–111, 111fstructure of, 110, 111f, 874, 874f synthesis of, 843, 869, 874–875, 875f, 876f
drugs affecting, 877 regulation of, 877
TCA cycle intermediates and, 471f, 472 Heme synthase, 875, 875f Hemicelluloses, 422f, 424t Hemoglobin, 15, 843, 872–874
adult, 80, 90, 108
buffer function of, 47, 54–55, 55f carbon dioxide binding in, 114, 114f conformation changes in, 123–124, 123f degradation of, 877–879, 878f disorders of, 884–886
embryonic, 885–886 fetal (HbF), 80, 90
hereditary persistence of, 870, 885–889, 890t glycosylated (A1C), 27, 56, 101, 120, 122, 379, 570, 863 glycosylation of, 119, 120f, 121
heme component of, 486, 487 iron component of, 486, 487, 874 in iron-deficiency anemia, 295 measurement of, 252, 436 myoglobin as paralog of, 89, 90f normal values of, 872, 872t oxygen binding in, 109–114, 111f agents affecting, 112–114, 113f
2,3-bisphosphoglycerate and, 113, 113f cooperativity of, 112, 112f, 123–124, 123f proton binding (Bohr effect) and, 113–114, 113f oxygen saturation curve for, 110, 110f, 112 polymorphisms in, 80
structure–function relationships in, 109–114 structure of, 109–110, 110f
synthesis of, 869
in thalassemia, 252, 267, 268
turnover of, 743
Hemoglobin A1c, 27, 56, 101, 120, 122, 379, 570, 863 Hemoglobin C (HbC), 884, 890t
Hemoglobin H (HbH), 884–885 Hemoglobin M, 487 Hemoglobinopathies, 884–886 Hemoglobin S (HbS, sickle cell), 884 abnormal protein structure in, 112
amino acid substitution in, 86, 87, 88, 90, 884 genetics of, 80
Hemoglobin switching, 869, 885–889, 886f, 888f Hemolysis, 874
Hemolytic anemia, 843, 872, 879, 884, 886–887 Hemophilia, recombinant factor VIII for, 319, 334 Hemophilia A, 894, 904–905, 907t
Hemophilia B, 905, 907t
Hemosiderin, 877, 877f
Hemostasis, 843–844, 870, 893, 895–904. See also Coagulation Hemostatic plug, 893, 895–897
formation of, 896–897
secondary, 893Henderson-Hasselbalch equation, 47, 52, 54 Henseleit, Kurt, 757
Heparin, 602, 903, 905, 985f, 986, 986t Heparin-induced thrombocytopenia (HIT), 905, 964 Heparin sulfate, 844, 903, 986, 986t, 992 Hepatic artery, 910, 911, 911f, 913
Hepatic binding protein, 914 Hepatic dysmetabolism, 702
Hepatic encephalopathy, 757, 760, 764, 766t
Hepatic fibrosis, 713–714, 713t, 714f, 715t, 926–928 Hepatic parenchymal cells. See Hepatocytes
Hepatic portal vein, 27, 29, 910, 911, 911f, 913 Hepatic sclerosis, 713t
Hepatic steatosis (fatty liver), 185, 603, 649, 702, 708 Hepatic triacylglycerol lipase (HTGL), 680–681, 680f, 684, 925 Hepatitis
alcohol-induced, 710, 711f vaccines against, 319 viral, 753, 757, 764, 766t Hepatitis A, 753, 757, 764 Hepatitis B, 341t, 362
Hepatitis B vaccine, 320, 333, 335, 338, 362 Hepatobiliary transport, 910
Hepatocellular carcinoma, 362 Hepatocytes, 27, 911–912, 911f, 912 diagnostic agents for, 914 functions of, 912
protein synthesis in, 919 receiving and recycling in, 913–914 secretions of, 911
Hepatogenous diabetes, 927 Heptahelical receptors, 190, 198, 198f nomenclature for, 203 phosphatidylinositol signaling by, 205 signal transduction through, 203–205 Heptoses, 66
Herceptin (trastuzumab), 349 Hereditary anemias, 843, 869
Hereditary fructose intolerance, 435, 442, 451–452, 454t Hereditary nonpolyposis colorectal cancer, 242, 247, 248t, 349, 363 Hereditary orotic aciduria, 816, 820t
Hereditary persistence of fetal hemoglobin (HPFH), 870, 885–889, 890t Herpesvirus 8 (HHV-8), 362
Hers disease, 531, 531t, 532 Heterochromatin, 181f, 214, 301