168397_P489-520.qxd7.0:34 Index 6-2-04 26p 2010.4.5 10:03 AM Page 489 source of, 109, 109f pairing with thymine, 396f, 397, 398f in tricarboxylic acid cycle, 109–111, 109f Adenine arabinoside (vidarabine, araA), 409 Acetyl CoA-ACP acetyltransferase, 184 Adenine phosphoribosyltransferase (APRT), Index Acetyl CoA carboxylase, 183, 185f, 190 296, 296f in absorptive/fed state, 324 Adenosine deaminase (ADA), 299 allosteric activation of, 183–184, 184f deficiency of, 298, 300f, 301–302 allosteric inactivation of, 183, 184f gene therapy for, 485, 486f dephosphorylation of, 184 Adenosine diphosphate (ADP) in fasting, 330 in ATP synthesis, 73, 77–78, 78f Page numbers in bold indicate main discus- hormonal regulation of, 184, 184f isocitrate dehydrogenase activation by, sion of topic. Page numbers followed by f long-term regulation of, 184 112 denote figures. “See” cross-references direct phosphorylation of, 183–184 transport of, to inner mitochondrial short-term regulation of, 183–184, 184f membrane, 79 the reader to the synonymous term. “See Acetyl CoA carboxylase-2 (ACC2), 191 in tricarboxylic acid cycle regulation, 114, also” cross-references direct the reader to N4-Acetylcytosine, 292f 114f related topics. [Note: Positional and configura- N-Acetyl-D-glucosamine, 142 in urea cycle, 255–256 N-Acetylgalactosamine (GalNAc), 160, 168 ribosylation, 95 tional designations in chemical names (for N-Acetylglucosamindase deficiency, 164f Adenosine monophosphate (AMP; also called example, “3-“, “α”, “N-“, “D-“) are ignored in N-Acetylglucosamine (GlcNAc), 160, 166, 168 adenylate) alphabetizing. N-Acetylglucosamine-6-sulfatase deficiency, cyclic. See Cyclic adenosine 164f monophosphate (cAMP) N-Acetylglutamate gluconeogenesis and, 123 A synthesis of, 255f, 256 glycogen degradation, 132, 133f Aβ, in Alzheimer’s disease, 21 in urea cycle, 253, 254f IMP conversion to, 295–296, 295f Abbreviations, for amino acids, 5, 5f N-Acetylglutamate synthase, 256 phosphofructokinase-1 activated by, 99 ABCA1, 236 N-Acetyllactosamine, 142 in urea cycle, 255–256 Abetalipoproteinemia, 231 CMP-N-Acetylneuraminic acid, 166 Adenosine triphosphate (ATP) Absorptive state, 321. See also Fed state N-Acetylneuraminic acid (NANA), 166 in aerobic glycolysis, 97–98, 100f, Acceptable Macronutrient Distribution Ranges in acidic glycosphingolipids, 209 102–104 (AMDR), 360, 360f synthesis of, 160 in amino acid deamination, 252 ACE. See Angiotensin-converting enzyme Acetyl residue, in plasma-activating factor, in amino acid transport, 249 Acetaldehyde, 317 202, 202f in anabolic pathways, 93 Acetanilid, 153 Acid/base properties in anaerobic glycolysis, 104 Acetate (acetic acid), 6f, 7, 182f of amino acids, 6–9 in catabolic pathways, 91, 93f in cholesterol synthesis, 220 Henderson-Hasselbalch equation for, 6–9 change in free energy of, 77 titration of, 6f, 7 Acidemia in cholesterol synthesis, 220 Acetoacetate, 195–196, 196f ketone bodies and, 197 as energy carrier, 72–73, 73f formation in amino acid catabolism, 261, methylmalonic, 194 in fatty acid synthesis, 183, 183f 262, 266, 266f Acid hydrolases, 162 in glycolysis, 96 Acetoacetyl CoA, formation in amino acid, Acidic amino acids, 3f, 5 in GMP synthesis, 295–296 262, 266 Acidic sugars hydrolysis of, standard free energy of, 73 Acetone, 195, 196f, 262 in glycosaminoglycans, 157, 157f, 161, isocitrate dehydrogenase inhibition by, Acetylation, 422, 422f 162f 112 Acetylcholinesterase synthesis of, 161, 162f in muscle contraction, 132 inhibition by insecticides, 62 Acid maltase, 130 as phosphate donor, 63, 73 in membrane protein anchoring, 206 Aciduria phosphofructokinase-1 inhibited by, 99 Acetyl CoA, 96 homogentisic, 274 production/synthesis of in absorptive/fed state, 323, 323f, 324 methylmalonic, 194 in fatty acid oxidation, 192, 192f allosteric activation of, 122 orotic, 303, 303f in inner mitochondrial membrane, 74 amino acids that form, 266 Acne, retinoic acid for, 385 in oxidative phosphorylation, 73, 73f, carboxylation to malonyl CoA, 183–184, Aconitase 77–80 184f in citrate isomerization, 111f, 112 by 3-phosphoglycerate, 101–102 in cholesterol synthesis, 220 inhibition of, 112 in pyruvate formation, 102–103 in citrate synthesis, 111–112, 111f Acquired hyperammonemia, 258 in tricarboxylic acid cycle, 109, 113, conversion of building blocks to, 93, 93f ACTH, 239, 239f 113f, 114f cytosolic, production of, 183 Active sites, 54, 54f, 56, 57 in protein degradation, 247 decarboxylation of pyruvate to, 96, 105, Acute intermittent porphyria, 280, 281f in protein synthesis, 437 106f, 109–111, 110f Acyl carrier protein (ACP), 184 structure of, 73, 73f in diabetes mellitus, 197 Acyl CoA:cholesterol acyltransferase (ACAT), transport to inner mitochondrial in fatty acid synthesis, 183–184 177, 233f, 234, 234f membrane, 79 formation of Acyl CoA dehydrogenase, 76 in urea cycle, 255–256 α β in amino acid catabolism, 261–262, Acyl CoA derivatives, - -unsaturated, 266 Adenylate kinase, 296, 296f 266, 266f Acyl CoA:diacylglycerol acyltransferase Adenylyl (adenylate) cyclase, 134, 151 in fatty acid oxidation, 192, 192f (DGAT), 176 Bordetella pertussis and, 95 in gluconeogenesis, 119, 119f, 122 Acyl CoA:monoacylglycerol acyltransferase glucagon and, 314 in glycolysis, 96 (MGAT), 176 lactose operon and, 451f, 452 in ketone body synthesis, 195–196, 196f Acyl CoA oxidase, 195 in metabolic regulation, 94–96, 95f oxidation of, 93, 93f Acyltransferases, 176–177 in triacylglycerol degradation, 190 pyruvate dehydrogenase activated by, Added sugars, 365 Vibrio cholerae and, 94 119, 119f Adenine, 291, 291f, 292f, 305f, 396, 396f, 397 Adequate Intake (AI), 358, 359f pyruvate dehydrogenase complex as in codons/genetic code, 431, 432f Adipocytes free fatty acids reesterified in, 178 489 168397_P489-520.qxd7.0:34 Index 6-2-04 26p 2010.4.5 10:03 AM Page 490 490 Index in obesity, 350–351 ALA synthase, 278 Prevention Trial, 391 volume of, 324f, 325 effects of drugs on, 279 Alternative splicing, 457, 457f Adiponectin in porphyrias, 280 Alzheimer disease, 21, 21f, 23f, 231 in diabetes mellitus, 343 Albinism, 263, 268f, 269f, 273, 273f, 288 Amanita phalloides, 251f, 424 in obesity, 353 oculocutaneous, 272, 272f Amidation, synthesis of nonessential amino Adipose tissue Albumin acids by, 268 in absorptive/fed state, 324–325 of aldosterone, 237 Amide group, in amino acid side chains, 4 carbohydrate metabolism in, 325, 325f of bile acid, 225 Amine oxidase, 257 fat metabolism in, 325, 325f of bilirubin, 282 Amine(s) intertissue relationships of, 328f of free fatty acids, 178, 190 ammonia from, 257 communication with other metabolic function of, 4 biologically active, 285–287 organs, 307, 307f Alcohol Amino acid analyzer, 15, 15f in diabetes mellitus coronary heart disease and, 364–365 D-Amino acid oxidase, 253 type 1, 339, 339f in cirrhosis of liver, 318 Amino acid pool, 246, 246f type 2, 343, 344 in fatty liver, 318 Amino acid(s), 1–12 in energy metabolism, 307, 307f in glycerophospholipids, 202 abbreviations and symbols for, 5, 5f as energy storage depot, 324–325 hypoglycemia and, 317–318, 317f absorption of, 249 in fasting, 327, 330f, 331, 331f metabolism of, 317–318, 317f in absorptive/fed state, 321 carbohydrate metabolism in, 331, 331f Wernicke-Korsakoff syndrome and, 379 acid/base properties of, 6–9 fat metabolism in, 331, 331f Alcohol dehydrogenase, 317 acidic, 3f, 5 intertissue relationships of, 328f Aldehyde dehydrogenase, 317 in α-helix, 16–17 fatty acids in, as fuel reserve, 189 Aldolase, 100 amino group of, 1, 1f, 4f, 7f, 8 hormones of, and obesity, 352–353 Aldolase A, 138, 138f ammonia from, 256 hyperplasia of, 351 Aldolase B, 138, 138f amphoteric properties of, 9 hypertrophy of, 351 deficiency of, 138 attachment to tRNA insulin resistance and, 343 Aldose reductase, 139, 140, 141f, 142 enzymes required for, 435, 435f lipoprotein lipase in, 228–229 Aldoses, 83, 83f site for, 434f, 435 metabolic role of, 307 Aldosterone, 237, 237f, 239, 240f basic, 3f, 5 in obesity, 324–325 Alkaline phosphatase, 206 branched-chain, 326 subcutaneous, 350 Alkaptonuria, 263, 268f, 269f, 274, 274f catabolism of, 266–267, 266f triacylglycerol fate in, 189 Alkyl group dehydrogenation of, 266–267 visceral, 350 saturated, in plasma-activating factor, oxidative decarboxylation of, 266 A-DNA, 398 202, 202f transamination of, 266 ADP. See Adenosine diphosphate unsaturated, in plasmalogens, 202, 202f as buffers, 6–7, 6f, 8, 9, 9f Adrenal cortex, cholesterol synthesis in, 220 Allantoin, 298 carbon skeletons of, 245, 250 Adrenal cortical steroid hormones Allele-specific oligonucleotide (ASO) probe, catabolism of, 262–267 deficiency of, 238f 472, 472f, 485f carboxyl group of, 1, 1f, 4f, 7 secretion of, 239, 239f Allolactose, 451f catabolism/degradation of, 245, 249–253, Adrenal medulla, tyrosine hydroxylase in, 286 Allopurinol, 301 261–267, 261f Adrenergic symptoms, of hyperglycemia, 315 Allosteric activators in absorptive/fed state, 323f, 324 Adrenocorticotropic hormone (ACTH), 239, in absorptive/fed state, 321, 321f acetoacetyl CoA formation in, 266 239f in fasting, 328 acetyl CoA formation in, 266, 266f Adrenoleukodystrophy, 195, 236 in metabolic regulation, 94 fumarate formation in, 263, 263f Afferent signals, 352–353, 353f Allosteric effectors, 27, 41f, 62–63, 62f α-ketoglutarate formation in, 250–252, African Americans in absorptive/fed state, 321, 321f 250f, 252f, 262, 263f lactose intolerance in, 88 carboxylation of pyruvate and, 119 oxaloacetate formation in, 262, 262f sickle cell disease in, 35 in fasting, 328 pyruvate formation in, 263, 263f Age-related macular degeneration