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for gluconeogenesis, 566, 570–571, 571f, 575 renal release of, 829

skeletal muscle, 445, 449, 451, 473, 945 TCA cycle and, 458, 470, 471, 472, 473 tissue sites of production, 444–445, 444t

Lactate dehydrogenase (LDH), 105, 105f, 107, 138–139, 138f in anaerobic glycolysis, 434, 444–445, 444f

in gluconeogenesis, 571, 571f subunits of, 445

Lactic acid, 47, 51t, 55 in dental caries, 444, 451

dissociation to lactate, 444 exercise and, 445, 449, 451, 473

production in anaerobic glycolysis, 444–445 in sweat, 445

Lactic acidosis (acidemia) congenital, 493

ethanol and, 702, 708, 710

glycolysis and, 434, 440, 444, 450, 452, 454t oxidative phosphorylation and, 492–493, 493f pathways leading to, 492, 493f

tricarboxylic acid cycle and, 458, 470, 472, 473 Lactobacilli, metabolism of, 186

Lactose, 5, 25, 69, 70f, 369, 418, 435 digestion of, 415, 419–421

structure of, 415, 416f

synthesis of, 371, 544, 553–554, 553f

Lactose intolerance, 417, 423–424, 429, 431t, 554, 555 Lactose synthase, 554

Laennec cirrhosis, 712, 926 Laetrile, 490

Lagging strand of DNA, 234, 235f, 238 Laminin, 978, 984

defects in, 984 integrins and, 989

structure of, 984, 984fsynthesis of, 984 Lamivudine, 234, 268, 269

Lanosterol, 672–673

Large external transformation-sensitive (LETS) protein, 990 Large neutral amino acids (LNAAs), 957–958

Laws of thermodynamics, 397t LBK1 protein, 714 L-configuration, 80, 82, 82f LDL. See Low-density lipoprotein LDL direct, 667

L-DOPA, 519, 961

Lead, inhibiting action of, 142–143 Leading strand of DNA, 234, 235f, 238

Leber hereditary optic neuropathy (LHON), 491t Lecithin. See Phosphatidylcholine

Lecithin–cholesterol acyltransferase (LCAT), 667, 682, 682f, 683, 683f, 924–925 Left ventricular heart failure (LVF), 395, 409

Leigh disease, 470, 472, 476t, 491t Leptin, 656–657, 660

Lesch-Nyhan syndrome, 806, 811, 819, 820t Leucine, 12, 83, 84f, 85t

conversion to glutamine, 830–832, 831f degradation of, 771, 782

disorders of, 786t functions of, 827–828

oxidation in skeletal muscle, 823, 825, 827, 830, 831f oxidation to acetyl coenzyme A, 469

principles governing interorgan flux, 827–828 Leucine zippers, 307, 307f, 673

Leukemia, 881

chronic lymphocytic, 360

chronic myelogenous, 295, 313–314, 315t, 345 interferon therapy for, 308, 362

lymp

hocyte percentage in, 295

Philadelphia chromosome in, 295, 303, 313–314, 345, 349, 362 stem cell transplantation for, 362

tyrosine kinase inhibitor for, 362 white blood cell count in, 295, 362 decreased platelets in, 345

lymp hoid, 883

myeloid, 883 T-cell, 362

Leukocyte(s), 869–871 anaerobic glycolysis in, 444

in chronic myelogenous leukemia, 295 classification and functions of, 870–871 integrins and, 989

morphology of nucleus, 870–871 Leukocyte adhesion deficiency (LAD), 989 Leukotrienes, 70, 194–195, 638

in gout, 195

lipoxygenase pathway for, 640, 642f structure of, 642f

synthesis of, 640, 642f Levodopa (L-DOPA), 519, 961 Levofloxacin, 282 LibrariesDNA, 327–329 genomic, 327, 328, 331 Life stage group, 11 Li-Fraumeni syndrome, 354 Ligand(s), 101, 105

for heme, 110–111

for immunoglobulins, 114 prosthetic groups, 110 quantitation of binding, 109

Ligand-gated channels, 175–176, 176f Ligases, 144, 146

Light (L) chains of immunoglobulins, 114–116, 115f Lignan, 424

Lignin, 418, 424t

Limit dextrins, 415, 419, 419f, 420, 421 Linagliptin, 863

Lineages, cell, 880

Lineweaver-Burk transformation, 153, 153f, 155, 156f Lingual lipase, 594, 595, 596f

Linoleate, 609, 632 oxidation of, 615–616, 615f Linoleic acid, 638, 641f, 669 Linolenic acid, 70, 71f, 638 Lipase

adipose triglyceride, 649–651, 650f gastric, 594, 595, 596f

hepatic triacylglycerol, 680–681, 680f, 684, 925 hormone-sensitive, 649, 650f, 660, 724

lingual, 594, 595, 596f

lipoprotein, 591–592, 594, 632, 632f, 649, 678, 723–724 apoCII activation of, 600–601, 601f, 678

defective or deficiency, 651, 652 in liver disease, 925 pancreatic, 594, 596–597, 597f

Lipid(s), 591–593. See also specific types addition of (fatty acylation), 92, 93f categories of, 591

elevated levels of, 29, 30, 31t, 634, 649, 651, 652, 658–659, 660–662 emulsification of, 25, 594, 596

ether, 181 hydrophobicity of, 62, 65 insolubility of, 591 melting point of, 69 membrane, 591

metabolic syndrome and, 660–662, 695–696 metabolism of (See Lipid metabolism)

micelle packaging of, 25–26, 72, 594, 597–598 myelin/myelin sheath, 953–954, 971–972 structure of, 62, 69–72

synthesis of, 591, 631, 631f, 720–723 in brain, 844, 970–972

in peripheral nervous system, 970–972 Lipid(s), dietary (fats), 594–604 absorption of, 25–26, 26f, 597–598

calories/energy yield from, 5t, 394, 406, 407, 595, 609 digestion of, 24, 25–26, 26f, 594–597

fuel stores from, 7, 24guidelines on, 17 requirements for, 11

transport in blood, 600–603 Lipid-anchored proteins, 174 Lipid bilayer, 169, 171–173, 171f

Lipid-lowering drugs, 197, 667–668, 668t, 696t for atherosclerosis, 688, 695–696

bile acid resins, 696t

ezetimibe, 669, 670, 695, 696, 696t fibrates, 696t, 698, 923

niacin, 659, 696t

statins, 659, 667–668, 668t, 670, 673, 688, 695–696, 696t, 915 Lipid metabolism, 591–593, 922–925

in brain, 953–954, 970–972 ethanol and, 702, 708–709, 709f

in fasting state, 34, 36–40, 39f, 566, 582, 593, 593f, 607, 623, 724–726 in fed state, 24, 24f, 29, 592, 592f, 720–724

general summary of, 726–728, 727f insulin in, 609, 724

integration with carbohydrate metabolism, 719–732 in liver disease, 924–925, 927

in peripheral nervous system, 970–972 Lipid peroxidation, 509–510, 509f, 510f ethanol and, 711–712

vitamin E as defense against, 517, 517f Lipid peroxides, 509

defense against, 505 formation of, 504, 506, 508 Lipid profile, blood, 25 Lipoate, 457, 461, 465, 465f Lipofuscin, 509

Lipogenesis, 38t, 591, 631, 631f. See also Fatty acid synthesis Lipolysis, 34, 36, 37, 608, 649–651, 650f. See also Lipid metabolism activation of, 609

mechanisms affecting, 724

Lipoprotein(s), 29, 591–593, 592t, 598–602, 895t. See also specific types cholecalciferol in, 666

cholesterol in, 598–599, 666–667, 678–684 elevated levels of, 600, 634, 658–659 fed state, 24–26, 27, 29

in liver disease, 925

protein constituents of, 599. See also Apolipoprotein(s) receptor-mediated endocytosis of, 178, 684–685, 684f structure of, 599f, 678

triacylglycerols in, 568, 591–592, 592f, 598–602, 632, 632f, 647–649 Lipoprotein a (Lip(a)), 679t, 690

Lipoprotein lipase (LPL), 591–592, 594, 632, 632f, 649, 678, 723–724 apoCII activation of, 600–601, 601f, 678

defective or deficiency, 651, 652 in liver disease, 925 Lipoprotein receptors, 685–687

Liposomes, as cloning vectors, 327 Lipostatin, 601

Lipoxins, 640

Lipoxygenase pathway, eicosanoid synthesis in, 640, 642f Liraglutide, 863

Lispro (Humalog), 91, 95, 335Lithocholic acid, 676, 678f Liver

as altruistic organ, 844 anatomy of, 911, 911f cell cycle in, 236

cell types in, 912–913

creatine synthesis in, 940f, 941

diseases of, 926–927 (See also specific diseases) alcohol-induced, 702, 708–714, 926–928

amino acid metabolism in, 926 blood glucose levels in, 927

fibrosis in, 713–714, 713t, 714f, 715t, 926–928 lipid metabolism in, 924–925, 927

as excretory organ, 910 fatty, 185, 603, 649, 702, 708 fatty acid synthesis in, 634 fuels for, 921–926

fatty acid, 624, 922–925 glucose, 921–922

functions of, 844, 910, 913–921 glucose transport in, 428

glycogen stores in, 7, 7t, 27, 371, 525, 527, 527f, 720 nitrogen-containing products of, 918, 919t

nitrogen transport to, 751, 756–757, 756f, 823 protein synthesis in, 38, 894, 895t, 918–919 purine biosynthesis in, 807, 811

as receiving and recycling center, 913–914 secretory systems of, 910, 911

size of, 910

strategic position of, 910 transport mechanisms of, 910, 914

triacylglycerol synthesis in, 29, 647–648, 647f, 720–723, 918 urea synthesis in, 823

VLDL synthesis in, 648, 648f

xenobiotic inactivation and detoxification in, 914–917 Liver-associated lymphocytes, 913

Liver failure, 712, 926, 929t

Liver metabolism, 38t, 726–728, 727f, 844, 910–928. See also specific processes and substances

amino acid, 29, 751, 752f, 770, 823, 833–834, 920, 925–926 fasting state, 826–827, 826f

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

bile salt, 675–678

carbohydrate metabolism in, 921, 921t cholesterol, 674–675, 918

fasting state, 34 brief fast, 35–37

glycogen metabolism in, 34–38, 532–533, 532t, 533t, 566, 581, 724–726 prolonged fast, 37–40

fatty acid, 34, 624, 634, 922–925 flowchart of changes in, 728t

glucose, 27, 34–38, 568–569, 579, 582f, 724, 917–918, 921–922 (See also Gluconeogenesis)

glycogen, 34–38, 526, 527, 527f, 531–537, 532t, 534f, 566, 720–723

blood glucose levels and, 535, 917–918 calcium and, 535–536, 536f

diabetes mellitus and, 535

epinephrine and, 534f, 535–537, 536f, 853, 854ffasting 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 growth hormone and, 851–853

ketone body production in, 724–726, 918 (See also Ketone bodies) NADPH demand in, 920–921

nitrogen conversion to urea in, 751, 918 (See also Urea cycle) regulatory enzymes of, 729t

TCA cycle in, 471–472, 471f thyroid hormone and, 859 xenobiotic, 914–917, 914f Liver transaminases, 609

ALT, 571, 571f, 609, 712–713, 755, 757, 926–927 AST, 609, 712–713, 755, 757, 926–927

in hepatitis A, 757 le

vels as sign of disease, 926–927 Liver X-activated receptor (LXR), 197 LKB1 protein, 697, 730

Lock-and-key model, 130–131

Locus control region (LCR), 887, 888f Long-chain acyl-CoA dehydrogenase (LCAD), 611t Long-chain fatty acids, 607

activation of, 610–611, 610f, 611t albumin binding of, 607, 608f, 610 β-oxidation of, 613–617, 922 cellular uptake of, 610

dietary, 609

fatty acyl coenzyme A from, 607, 608f, 610–612, 610f as fuel, 609–612

hydrophobic nature of, 610 metabolism of, 607, 608f transport of, 610–612, 612f

Long interspersed elements (LINEs), 266–267 Loop conformation, 105

Loose connective tissue, 978 Loss-of-function mutations, 344 Lou Gehrig disease (ALS), 515, 522t

Low-density lipoprotein (LDL), 29, 592, 592t, 666, 680–681 characteristics of, 679t

elevated levels of, 634, 649, 658–659, 667–668, 686, 687, 689 feedback inhibition of, 685

formation of, 680f, 681 in liver disease, 925

measuring blood concentration of, 667 receptor-mediated endocytosis of, 684–685, 684f

Low-density lipoprotein (LDL) receptor, 666, 685–687, 686f, 689 Low-density lipoprotein (LDL) receptor-related protein (LRP), 685, 687 Low-molecular-weight (LMW) heparin, 905

L-sugars, 62, 66–67, 67f Luminal agent, 914

Lung cancer, 231, 246, 248t

adenocarcinoma in, 231, 345, 346deaths from, 361, 361f development of, 361

metastatic, 345, 363

smoking and, 231, 240, 246, 361

TNM staging in, 363

Lung surfactant, 651, 655, 659

Lupus, 252, 262, 269, 271t, 979, 990–991, 994t Lutein, 518

Luteinizing hormone, 693–694 Lyases, 144, 145f, 146 Lymph, 594

chylomicron secretion into, 594, 600 Lymphocytes, 871

base salvage in, 811

in chronic myelogenous leukemia, 295 liver-associated, 913

normal values of, 870t production of, 881f Lymphoid leukemia, 883 Lymphoma

Burkitt, 348

follicular, 295, 313, 315t, 360, 482 non-Hodgkin, 295, 313, 315t doxorubicin toxicity in, 482 HIV-related, 362

interferon therapy for, 308 methotrexate for, 303, 795, 796 miRNA expression in, 313

R-CHOP chemotherapy for, 295, 313, 819 Lymph system, 594

Lysine, 12, 84f, 85t, 86, 86f degradation of, 771, 771f, 782–783

residues, in collagen, 979–980, 980f, 981f Lysis, definition of, 614 Lysophospholipids, 594

Lysosomal α-glucosidase, 530, 531t Lysosomal granule, 896

Lysosomal hydrolases, 179, 179f Lysosomal proteases, 738

Lysosomal storage diseases, 179, 954 Lysosomes, 169, 170f, 178–181 formation of, 179

hepatic, 910

in phagocytosis, 180

protein turnover in, 738, 744 proteoglycan degradation in, 978, 988 reactions of, 179f

in receptor-mediated endocytosis, 179–180 residual body, 180

MMa

croadenoma, 849, 850, 851 Macrocytic anemia, 872, 872t Macrolide antibiotics, 275, 283 Macrominerals, 13–15 Macronutrients, 3, 4, 11

Macrophages, 871in atherosclerosis, 688–689 phagocytosis by, 180

production of, 881f

Macrophage scavenger receptor, 685, 687 Macular carotenoids, 518

Macular degeneration, age-related, 518, 522t Mad cow disease, 119–121

Magnesium

in catalysis, 139 function of, 15

in tricarboxylic acid cycle, 459 Major groove, of DNA, 218, 219f Malaria

sickle cell anemia and, 90

thalassemia and, 884 Malate

oxaloacetate interconversion with, 573, 573f precursors of, 469

Malate–aspartate shuttle, 434, 434f, 443f, 488–489, 488f

Malate dehydrogenase, 459, 461, 467, 474, 635, 635f. See also Malic enzyme Malathion, 129, 129f, 140, 143, 143f, 147t

Malic enzyme, 631, 635, 635f, 721, 722f, 723, 729t, 828 Malignant tumors (neoplasms), 212, 214, 344, 345, 346 Malnutrition, 13, 42t

ATP generation in, 375

depression associated with, 4, 14, 19, 35, 37, 40 kwashiorkor, 13, 20t, 423, 740, 748t, 894, 907t maternal, and neonatal hypoglycemia, 526, 532, 538 midarm anthropometry in, 30

protein, 13, 423, 583, 739, 740, 745–746, 748t, 835, 894 protein-energy, 10, 13, 35

protein synthesis in, 38 severe acute, 13 starvation and, 34, 37–40

Malondialdehyde, 504, 509, 510f

Malonyl coenzyme A (malonyl-CoA), 721–723, 722f

CPTI inhibition by, 617, 618f, 637, 639f, 722, 723f, 932, 938 cytosolic acetyl-CoA conversion to, 636, 636f, 721–723

in fatty acid elongation, 637, 640f

in fatty acid oxidation, 607–608, 617, 618f, 624, 625f in fatty acid synthesis, 631, 636–637, 721–723

in hepatic fibrosis, 714

in ketone body synthesis, 624, 625f, 724 in muscle metabolism, 932, 938, 941–942

Malonyl coenzyme A (malonyl-CoA) decarboxylase (MCD), 730, 932, 938, 938f, 939 Maltose, 5, 415, 419f, 421, 425

Maltotriose, 415, 420f, 421

Mammalian target of rapamycin (mTOR), 730, 731f, 744, 744f, 837–838 Mammary glands, milk production in, 596

Mannose, 544, 554, 556–558

Mannose 6-phosphate, 179, 180, 554, 556–558 Maple syrup urine disease, 781, 787t, 958 Marasmus, 10, 13, 20t

Marfan syndrome, 986

Masculinization (virilization), 668, 690, 694, 696–697 Mast cells, 964

Matrixextracellular (See Extracellular matrix) mitochondria, 180, 180f

Matrix metalloproteinases (MMPs), 743t, 928, 978, 990 assay for, 990

tissue inhibitors of, 978, 990 Mature chylomicrons, 594, 600, 600f

Maturity-onset diabetes of the young (MODY), 153, 166t, 384, 390t McArdle disease, 531, 531t, 532

McClintock, Barbara, 245 mdm2 gene, 354

Mean corpuscular volume (MCV), 872 Measles, 284

Mechanical work, energy transformation for, 394, 399, 399f Mechanism-based inhibitors, 128, 140–143

Medial Golgi stacks, 182

Medium-chain acyl-CoA dehydrogenase (MCAD), 611t

Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency, 608–609, 615, 617, 625–626, 628t

Medium-chain fatty acids, 607 activation of, 611t

dietary, 609 metabolism of, 607, 609

oxidation of, 611t, 617, 922–923

Medium-chain-length acyl-CoA synthetase, 617

Medium-chain-length fatty acid-activating enzyme (MMFAE), 923, 924 Medium-chain-length triacylglycerols (MCTs), 922–923 Megakaryocytes, 871, 881f, 896

Megaloblastic anemia, 14t, 790–791, 792, 796, 803t, 883 “Megaloblastic madness,” 799

Meiosis, crossing over in, 243 Meischer, Frederick, 215 Melanin, 346

Melanin deficiency, 962 Melanocytes, 346

Melanoma, 231, 246, 248t, 345, 346 biopsy for, 231, 246, 345, 363 genetics of, 353

survival and surveillance in, 240 warning signs in, 363

Melatonin, 519, 963

Melting, DNA, 220

Melting point, of fatty acids, 69

Membrane(s), 170–171. See also specific membranes Membrane proteins, 171–174

of erythrocytes, 879–880, 880f integral, 169, 171–172, 171f, 173, 173f peripheral, 171–172, 171f, 173f, 174 Membrane skeleton, 169

Mercury, inhibiting action of, 142 Mesangium, 992, 992f

Messenger(s). See also specific messengers chemical, 190–208

second, 159, 190–191 Messenger RNA (mRNA), 211, 213

cap of, 213, 224, 224f, 251, 259–260, 260f, 310 coding region of, 213, 224, 224f

codons of, 211, 213, 224, 224f, 254, 254f

degradation of, regulation of, 313, 313feukaryotic, 224, 224f, 267t insulin and, 389

mitochondrial, 277

poly(A) tail of, 213, 224, 224f, 251, 259–261, 259f, 261f, 310, 310f posttranscriptional processing of, 310–313

precursors of (pre-mRNA), 224, 251, 259 prokaryotic, 267t

reading frame of, 274, 277, 277f stability of, 313

structure of, 224 synthesis of

in bacteria, 251, 258

in eukaryotes, 251, 259–261, 259f, 260f intron removal in, 261, 261f, 262f promoter regions for, 255–257, 256f

translation of, 91, 161, 211, 211f, 213, 274–290, 311–313 (See also Translation) transport of, 313

Metabolic acids, 47, 51, 53–56, 155 Metabolic bases, 47

Metabolic encephalopathies, 968–970 Metabolic homeostasis, 377–380, 378f definition of, 377

glucose’s role in, 378–379 intertissue integration in, 377

major hormones of, 379–380, 379f, 380f (See also Glucagon; Insulin) signals regulating, 378, 378f

Metabolic neuropathies, 968–970

Metabolic pathways, 1, 2f, 719. See also specific pathways allosteric enzymes in, 158

bacterial, 185–186 hepatic

flowchart of changes in, 728t general summary of, 726–728, 727f regulatory enzymes of, 729t

integration of carbohydrate and lipid metabolism, 719–732 multiple control points in, 719

multiple regulators of, 719 regulation of, 150, 151, 162–164 compartmentation in, 150, 151, 163–164 complexity of, 164

counterregulation of opposing pathways, 163 feedback, 150, 151, 162f, 163 feed-forward, 150, 151, 163

function matching in, 162 principles of, 162, 446–447, 447t

rate-limiting step in, 150, 150f, 162–163 second principle of, 164

Metabolic rate basal, 8–9, 374 resting, 8, 408, 496

Metabolic requirements, basic, 1

Metabolic syndrome, 30, 660–662, 667, 695–698 Metabolite measurements, 41

Metachromatic leukodystrophy, 559t Metal-ion catalysis, 133, 139 Metanephrine, 963

Metastasis, 212, 214, 226, 344, 346, 978, 986, 990. See also specific typesMetathyroid diabetes mellitus, 860

Metformin, 697–698, 698f, 863 Methemoglobin, 869, 874 Methemoglobinemia, congenital, 874, 890t Methemoglobin reductase, 874 Methionine, 12, 84f, 85t, 86

cysteine synthesis from, 774–775, 775f degradation of, 771, 776, 779–781, 779f disorders of, 777, 779, 780, 784–785 homocysteine from, 775, 775f, 790

as one-carbon-group source, 795t regeneration from homocysteine, 779, 790, 799 SAM formation from, 779, 790, 799

vitamin B12 and formation of, 790, 798, 798f Methionyl-tRNA, 274, 278, 279–281, 280f Methotrexate, 303, 795, 796

Methylation amino acid, 284 DNA

error in, 242–243

in gene expression regulation, 294, 301, 302 Methyl-B12 deficiency, 779

Methyl group, 63

Methylmalonyl coenzyme A, 571, 616, 616f

amino acid degradation to, 771f, 779, 779f, 780, 781 conversion to succinyl-CoA, 571, 616, 779, 779f, 790, 798, 798f propionyl-CoA conversion to, 571, 616, 779, 779f

Methylmalonyl coenzyme A mutase, 970 Methylprednisolone, 568 Methyltetrahydrofolate deficiency, 779 Methyl-trap hypothesis, 790–791, 799 Metronidazole, 914

Metropolitan Height and Weight Tables, 12 Mevalonate, 670–671

conversion to activated isoprenes, 670–671, 671f synthesis from acetyl-CoA, 670, 670f

MI. See Myocardial infarction Micelles, 25–26, 72, 594, 597–598

Michaelis-Menten equation, 150, 152–153, 152f, 165

Microarrays (chip), 319, 332–333, 362 Microclots (microthrombi), 897 Microcytic anemia, 872, 872t Microfibrils, 983

Microglial cells, 953, 956 Microminerals, 13–15 Micronodular cirrhosis, 926

MicroRNAs (miRNAs), 225, 312–313, 312f, 335 and apoptosis, 360

in follicular lymphoma, 313

and neural tube defects, 802–803 as oncogenes, 349

Microsomal, definition of, 182 Microsomal enzymes, 916

Microsomal ethanol oxidizing system (MEOS), 151, 154, 161, 164, 182, 184, 704, 705– 707, 705f, 708

Microsomal triglyceride transfer protein (MTP), 602–603, 603f, 648 Microsomes, 182Microtubule(s), 169, 170f, 182–183, 183f Microtubule-associated proteins (MAPs), 183

Microvascular disease, diabetic, 585, 979, 991–992 Microvilli (brush border), 415

Midarm anthropometry (AMC), 30

Mid-upper arm muscle circumference (MUAMC), 30 Mifflin-St. Joer equation, 9t

Milk, human, 596 Milliosmoles (mOsm), 50 Mineral(s), 3

dietary guidelines on, 18

dietary requirements for, 13–16, 16t excess intake of, 16 Mineralocorticoids, 689–692, 690t, 693 Minor groove, of DNA, 218, 219f Misfolding, protein, 119–121

Mismatch repair, 242–243, 243f Missense mutations, 277t, 278 Mitochondria, 169, 170f, 180–181

ATP generation in, 169, 180, 374, 374f, 480–481 and cell death, 358, 358f, 481, 482, 499 cellular respiration in, 374, 374f

enzyme compartmentation in, 474–475 enzymes of, 180

ethanol metabolism in, 702 hepatic, 910

la

ck in red blood cells, 28, 35, 444–445

oxidative phosphorylation in, 480–481, 480f (See also Oxidative phosphorylation) structure of, 180, 180f

swelling of, 482 TCA cycle in, 457 transport across

electron-transport chain for, 480–489 (See also Electron-transport chain) inner membrane, 180, 475, 475f, 496–498

lo

ng-chain fatty acid, 612, 612f

mitochondrial permeability transition pore for, 410f, 481, 497–498, 498f NADH shuttle systems for, 434, 434f, 443–444, 443f, 488–489, 488f

outer membrane, 180, 475, 475f, 497–498 proton leak in, 481, 494, 496

TIM complex for, 475, 475f TOM complex for, 475, 475f

Mitochondrial DNA, 180–181, 214 disorders of, 181, 500t

OXPHOS diseases, 481, 490–492, 491t heteroplasmy of, 491

mutations and inheritance of, 491