Index

ABC transporters

bile acid transport 11.5.3

deficiencies

Dubin-Johnson syndrome 11.5.4

sitosterolemia 11.8.3

Tangier disease 11.8.2

sterol transport 11.4.3, 11.4.7

structure and function 11.4.511.4.6

ACAT see acyl-CoA cholesterol acyltransferase

acetaminophen, metabolism 18.3.3

acetoacetate

decarboxylation 10.4.2

leucine degradation 12.4.4

mitochondrial acetyl-CoA export 10.5.7

synthesis 10.4.1

acetone

anticonvulsant activity 10.4.4

metabolism 10.4.210.4.3

substrate for gluconeogenesis 10.4.3

acetyl-CoA

formation by pyruvate dehydrogenase 5.2.1, 5.2.7

glyoxylate cycle 10.610.6.1

mitochondrial export 10.5.610.5.7

oxidation see citric acid cycle

acetyl-CoA carboxylase 10.5.1

acetylphosphate 3.3.8

aciclovir 16.9.1216.9.13

acid maltase 8.3.7

deficiency 8.6.2

enzyme therapy 19.3.219.3.3

acid-base catalysis 3.3.33.3.4

activation energy (ΔG*) 2.3

active transport

glucose 1.6.13

acute intermittent porphyria 17.3.5

acute myeloic leukemia (AML) 16.9.9

acute urate nephropathy 16.6.616.6.7

acyl carrier protein footnote 61

acyl thiokinase 10.3.2

acyl-CoA cholesterol acyltransferase 11.4.3

acyl-CoA dehydrogenase 10.3.3

acyl-CoA synthetase see acyl thiokinase

adenine phosphoribosyltransferase (APRT) 16.5.6

adenosine deaminase 16.5.1

adenosine deaminase deficiency 16.6.1

bone marrow transplant 19.2.1

enzyme therapy 19.2.319.2.4

experimental drug treatment 19.2.2

gene therapy 19.2.5

pathogenesis 19.2

adenosine kinase 16.5.6

adenosine monophosphate (AMP) deaminase 16.5.1, 16.5.2

adenosine monophosphate (AMP) synthesis 16.3.6

adenylate cyclase 7.5.4, 10.3.7

inhibition by metformin 14.5.14

adenylate kinase 2.5.12.5.2, 16.6.4

adenylosuccinate lyase 16.3.3, 16.3.6

AICAR see purine nucleotide synthesis

albumin

osmotic activity 10.2.4

transport of free fatty acids 10.3.7

alcohol dehydrogenase 2.2.4, 7.4.2

acetone breakdown 10.4.3

aldehyde dehydrogenase 7.4.2

acetone breakdown 10.4.3

aldolase 3.2

aldolase B 4.2.1, 16.6.4

deficiency (fructose intolerance) 4.2.2

aldose reductase 4.4

inhibitors, in diabetes 14.5.7

allantoin 16.5.5

allopurinol 16.6.5, 16.9.6

amino acids

conjugation 18.3.7

degradation

asparagine 12.4.1

glycine 15.2.6

histidine 15.2.7

leucine 12.4.4

overview 12.1.112.1.2

phenylalanine, tyrosine 12.4.5

serine 12.4.212.4.3, 15.2.415.2.5

metabolic uses 12.1

transamination

mechanism 12.2.112.2.2

overview 12.2

aminoacrylate 12.4.2

5′-aminoimidazole ribotide synthetase 16.3.3

aminoimidazole-carboxamido-ribotide (AICAR) transformylase 16.3.4

aminooxyacetate 6.9.2

aminotransferases see amino acids, transamination

ammonia

carbamoylphosphate synthesis 12.3.1

nitrogen fixation 1.4

release and scavenging in the liver lobule 12.3.8

urinary excretion footnote 73

AMP-activated kinase 14.5.14

amphibolic

definition 1.3

amylase 1.6.12

amylopectin 1.6.11

amylose 1.6.11

anabolic

definition 1.3

anaerobic glycolysis see glycolysis, anaerobic

anemia

hemolytic, in glucose-6-phosphate dehydrogenase deficiency 9.4

microcytic hypochromic 17.3.1

anorexia nervosa 16.6.3

apolipoproteins 11.4.1

apoptosis 18.5.1, 19.2, footnote 32

araC see cytosine arabinoside

archaebacteria 1.4

arginase 12.3.2

arginine deficiency, in ure cycle enzyme defects 12.3.10

argininosuccinase 12.3.2

argininosuccinate synthetase 12.3.2

asparaginase 12.4.1

asparagine synthetase 12.4.1

aspartate aminotransferase 6.9.2, 12.3.3

aspartate transcarbamylase 16.7.1

ataluren 19.1.319.1.4

atherosclerosis

and hypercholesterolemia 11.611.6.1

blood clot formation 11.6.2

metabolic aspects 11.6.5

role of oxidized LDL 11.6.12

vascular lesions

morphology 11.6.211.6.3

pathogenesis 11.6.4

atorvastatin 11.7.2

ATP

hydrolysis 3.3.1

role in enzyme reactions 2.42.4.1

ATP synthase

binding-change model 6.8.26.8.3

driven by proton flux 6.8.36.8.5

structure 6.8.16.8.2

ATP-citrate lyase 10.5.6

ATP/ADP transporter, mitochondrial 6.11.2

autotrophic

definition 1.4

bacteriorhodopsin 6.3.3

baker’s yeast 3.4.3

Banting, Frederick 13.2.3

benzbromarone 16.6.5

Best, Charles 13.2.3

β-alanine 16.7.3

β-oxidation

reaction mechanisms 10.3.410.3.5

reactions 10.3.3

β-hydroxybutyrate

synthesis 10.4.1

Bethune, Norman 13.2.4

bezafibrate 11.7.7

bile acids 1.6.8

absorbers, for lowering cholesterol 11.7.5

enterohepatic cycle 11.5.211.5.3

structure 11.5.1

bilirubin

conjugation and deconjugation 17.4

photoisomerization 17.4.4

bilirubin encephalopathy 17.4.3

biliverdin 17.4

biotin

leucine degradation 12.4.4

malonyl-CoA carboxylase 10.5.1

pyruvate carboxylase 7.2.3

1,3-bis-phosphoglycerate 3.2, 3.3.8

branched chain α-keto acid dehydrogenase 12.4.4

branched chain amino acid transaminase 12.4.4

branching enyzme 8.3.2

5-bromouracil-deoxyriboside (5-BUdR) 16.9.4

brown fat tissue 6.3.1, 10.3.8

C-peptide 13.2.6

canfosfamide 18.5.4

carbamoylphosphate synthetase

pyrimidine nucleotide synthesis 16.7.1

urea cycle 12.3.1

carbon monoxide, putative signaling function 17.4.6

4′-carboxy-5′-aminoimidazole ribotide carboxylase 16.3.3, 16.3.5

carboxyphosphate 7.2.3

carnitine 10.3.1

carnosine 16.7.4

catabolism

definition 1.3

overview 1.5.1

catalytic triad see chymotrypsin, catalytic mechanism

cataract

in diabetes 4.4

in type II galactosemia 4.3.4

CB 1954 18.5.3

cerulenine 10.5.9

cholecalciferol 11.2.7

cholesterol

biological functions 11.1

intestinal uptake 11.4.3

therapeutic inhibition 11.7.3

metabolism, as a therapeutic target 11.711.7.1

synthesis

overview 11.2.1

reactions 11.2.211.2.6

regulation 11.311.3.3

transport by lipoproteins 11.4.711.4.9

turnover, processes involved in 11.1.1

cholesterol ester transfer protein 11.7.4

cholestyramine 11.7.5

chylomicrons

disposal 10.2.6

synthesis and transport 10.2.310.2.5

vs. other lipoproteins 11.4.2

chymotrypsin

catalytic mechanism 2.22.2.2

cidofovir 16.9.14

citrate isomerase 5.4.3

citrate synthase 5.4.15.4.2

mitochondrial acetyl-CoA export 10.5.6

citric acid cycle 5.45.6

overall reaction 5.4.1

reactions 5.4.15.4.3

regulation 5.5

replenishment of intermediates 5.55.6

citrulline

nitric oxide synthase 9.3.5

urea cycle 12.3.2

cobalamin

deficiency

causes 15.5.5

treatment 15.5.5

in cyanide poisoning 15.5.5

intestinal uptake 15.5.4

methionine synthesis 15.4.2

structure 15.4.1

coenzyme Q 6.4, see also Q cycle

coenzymes

role in catalysis 2.2.3

coproporphyrinogen III 17.2.6

Cori cycle 8.5.3

covalent catalysis 3.3.43.3.5

coxsackievirus, and type 1 diabetes 14.4

Crigler-Najjar syndrome 17.4.2

Crohn’s disease 15.5.5

cyanide poisoning footnote 95

cobalamin treatment 15.5.5

cyclic AMP (cAMP)

dysregulation in diabetes mellitus 14.3.1

regulation of gluconeogenesis 7.5.37.5.5

regulation of glycogen metabolism 8.4.2

cyclic GMP (cGMP) 9.3.6

cystic fibrosis 19.1.4

cytarabine see cytosine arabinoside

cytochrome C 6.4, 6.4.2, 6.6.3

cytochrome C oxidase 6.4

reaction mechanism 6.6.36.6.4

cytochrome P450

acetone metabolism 10.4.3

in drug metabolism 18.218.2.6

prominent role of CYP3A4 18.2.2

structure 18.2.3

toxification of benzopyrene 18.2.7

transcriptional induction 18.2.2

cytochrome P450 reductase 18.4

cytosine arabinoside 16.9.716.9.10

dalcetrapib 11.7.4

debranching enzyme 8.3.5

7-dehydrocholesterol 11.2.7

ΔG and ΔG*, in enzyme reactions 2.3

δ-aminolevulinate synthase 17.2.2

5-deoxyadenosine 19.2.2

deoxycytidine kinase 19.2.2

deoxyribonucleotide synthesis 16.816.8.1

deoxyribose phosphate aldolase 16.4.2

desaturases 10.5.8

dexamethasone 13.3.7

diabetes

type 2

sulfonylurea drugs 13.2.11

diabetes insipidus footnote 82

diabetes mellitus

aldose reductase inhibitors 14.5.7

cataract 14.5.7

clinical symptoms 14.3.6

complications

long term 14.5.7

short term 14.5.6

dysregulation of glucose metabolism 14.3.1

dysregulation of lipid and protein metabolism 14.3.214.3.4

etymology 14.1.1

forms 14.1.2

hyperglycemia 14.5.6

hypoglycemia 14.5.6

insulin therapy, intensive 14.5.914.5.11

laboratory findings 14.3.5

oral antidiabetics 14.5.1214.5.13

renal glucose loss 14.2.6

therapy

acute case 14.5.1

type 1, pathogenesis 14.414.4.3

diaphorase 18.4, 18.5.3

dideoxyadenosine 16.9.11

digestion

bile, role of 1.6.8

enzymes 1.6.7

overview 1.5.1

digestive system 1.61.6.14

dihydrofolate reductase 16.9.5

inhibitors

antimicrobials 15.3

reactions 15.2.2

dihydroorotate 16.7.1

dihydroxyacetone phosphate 3.2, 4.2.1

glycerophosphate shuttle 6.9.3

1,25-dihydroxycholecalciferol 11.2.7

dimethylallyl-pyrophosphate 11.2.3

dinitrophenol 6.3.16.3.2

diphosphomevalonate decarboxylase 11.2.2

DNA polymerase, inhibitors 16.9.1016.9.14

DNA topoisomerase 16.9.10

drug metabolism

active metabolites 18.2.6

conjugation reactions 18.318.3.7

overview 18.118.1.1

phase I, II, and III 18.1.3

reductive 18.4

Dubin-Johnson syndrome 11.5.4

earthquakes 11.2.7

eliglustat 19.3.8

elongases 10.5.8

endoplasmic reticulum

smooth

sterol metabolism 11.2.8

energy-rich bonds and functional groups 3.3.73.3.8

enolase 3.2

enoyl-CoA hydratase 10.3.3

enoylreductase see fatty acid synthase, reactions

enzyme activity

allosteric regulation 2.5.4

and cooperativity 2.5.52.5.6

control by phosphorylation 2.5.5

transcriptional control 2.5.72.5.8

enzyme defects see also specific enzymes

enzyme therapy

adenosine deaminase deficiency 19.2.319.2.4, 19.3.619.3.7

Pompe disease 19.3.219.3.3

gene therapy

adenosine deaminase deficiency 19.2.5

technical considerations 19.1.5

lysosomal, enzyme therapy 19.319.3.7

therapeutic options 19.1.119.1.2

enzymes, IUBMB classification 2.2.4

epinephrine

gluconeogenesis 7.5.37.5.4

glycogen metabolism 8.4.2

hormone-sensitive lipase 10.3.7

signaling mechanism 13.3.113.3.3

epoxide hydrolase 18.3.2

equilibrative nucleoside transporter (ENT) 16.9.8

erythromycin 18.2.3

erythrose-4-phosphate 9.2.2

essential fatty acids 10.5.8

ethanol

degradation 7.4.2

folate deficiency 15.5.2

ethanolic fermentation 3.4.3

ezetimibe 11.7.3

Faraday’s constant 6.5.2

farnesyl-pyrophosphate

cholesterol synthesis 11.2.4

fast acetylators 18.3.6

fat tissue

white and brown 10.3.8

fatty acid synthase

pharmacological inhibition 10.5.910.5.10

reactions 10.5.410.5.5

structure and catalytic activities 10.5.210.5.3

fatty acids

common variants 10.2.1

elongation and desaturation 10.5.8

essential 10.5.8, footnote 51

mitochondrial transport 10.3.110.3.2

synthesis

overview 10.5

reactions 10.5.110.5.5

utilization 10.310.3.3

odd-numbered chain lengths 10.3.6

favism 9.49.4.4

ferritin 17.5.117.5.3

ferrochelatase 17.2.7

flavin adenine dinucleotide (FAD) 5.4.3, 6.4.2

flavin mononucleotide (FMN) 6.4.2

flavine adenine nucleotide (FAD) 10.3.3

5-fluorouracil 16.9.116.9.3

foam cells 11.6.3

folic acid

antimetabolites

antimicrobials 15.3

deficiency 15.5.215.5.3

uptake and storage 15.5.1

foodstuffs

energy contents 10.1.1

types 1.5

formic acid 4.3.3

formylglycinamidine ribotide synthetase 16.3.3

foscarnet 16.9.14

free energy (ΔG) 2.3

fructokinase 4.2.1, 16.6.4

fructolysis see fructose degradation

fructose

and obesity 4.2.1

degradation 4.24.2.1

hyperuricemia 16.6.4

in sperm fluid 4.4

intolerance 4.2.14.2.2

fructose-1-phosphate 4.2.1

fructose-1,6-bisphosphatase 7.2.57.2.6

putative role in hexose monophosphate shunt 9.2.2

regulation 7.5.3

fructose-1,6-bisphosphate 3.2

fructose-2,6-bisphosphate

regulation of gluconeogenesis 7.5.37.5.5

fructose-6-phosphate 3.2

hexose monophosphate shunt 9.2.2

β-fructosidase 4.2

fumarase 5.4.3

fumarate

citric acid cycle 5.4.3

futile cycles see substrate cycles

G protein coupled receptors 13.3.113.3.3

galactitol 4.4

galactokinase 4.3.1, 4.3.4

galactose

degradation 4.34.3.2

enzyme defects see galactosemia

galactose epimerase

reaction mechanism 4.3.14.3.2

galactose-1-phosphate 4.3.1, 4.3.4

galactose-1-phosphate uridyltransferase 4.3.1, 4.3.4

galactosemia 4.3.34.3.4

β-galactosidase 4.3, 4.3.3

γ-aminobutyrate (GABA) 17.3.5

ganciclovir 16.9.12

gastric acid 1.6.5

gastric lipase 10.2.2, 10.3.9

Gaucher disease 19.3.619.3.8

drug treatment 19.3.8

geranyl-pyrophosphate synthase 11.2.3

Gilbert’s syndrome 17.4.2

glucacon

signaling mechanism 13.3.113.3.3

glucagon 13.2.1

gluconeogenesis 7.5.37.5.4

glycogen metabolism 8.4.2

hormone-sensitive lipase 10.3.7

glucocerebrosidase 19.3.619.3.7

partial deglycosylation for enzyme therapy 19.3.7

glucocorticoids

metabolic effects 13.3.6

receptor agonists and antagonists 13.3.7

signaling mechanism 13.3.413.3.5

glucokinase 3.5.23.5.3

gluconeogenesis

energy balance 7.2.67.3

inhibition by ethanol 7.4.2

metabolic connections 7.37.4

mitochondrial transport in 7.4.1

overview 7.1.17.1.2

reactions 7.1.27.2.6

regulation 7.57.5.6

hormones 7.5.37.5.4

gluconolactonase 9.2.1

glucose

active transport 1.6.13

anomers 3.1.2

degradation

ATP yield 6.10.46.11.2

overview 3.13.1.1

GLUT transporters 1.6.13

regulation by insulin 13.2.16

subtypes and kinetics 3.53.5.2

intestinal uptake 1.6.13

organ-specific utilization 3.53.5.3

glucose alanine cycle 12.3.5

glucose-1-phosphate 4.3.1, 8.3.5

glucose-6-phosphatase 7.2.57.2.6

deficiency 8.6.1

expression in muscle 8.5.2

glycogen degradation 8.3.5

regulation 7.5.2

glucose-6-phosphate 3.2

glycogen degradation 8.3.5

hexose monophosphate shunt 9.2.1

in galactose degradation 4.3.1

glucose-6-phosphate dehydrogenase

deficiency 9.49.4.4

neonatal jaundice 17.4.5

reaction 9.2.1

glucosylceramide 19.3.6

GLUT transporters see underglucose

glutamate

in nitrogen disposal 12.3.7

in transamination 12.2

malate-aspartate shuttle 6.9.2

glutamate dehydrogenase 12.3, 12.3.6

glutaminase 12.3.712.3.8

regulation 12.3.9

glutamine

as a nitrogen carrier 12.3.6

glutamine synthetase 2.4.1, 12.3.712.3.8

glutamine-PRPP-amidotransferase 16.3.2

glutathione

peroxidase 9.4.2

scavenging of reactive oxygen species 9.3.8

glutathione-S-transferase 18.3.2

glyceraldehyde 4.2.1

kinase 4.2.1

glyceraldehyde-3-phosphate 3.2

hexose monophosphate shunt 9.2.2

glyceraldehyde-3-phosphate dehydrogenase 3.2

in apoptosis footnote 32

reaction mechanism 3.3.43.3.5

glycerol 4.2.1

glycerol-1-phosphate 4.2.1

glycerophosphate shuttle 6.9.26.9.3

glycinamide ribotide synthetase 16.3.2

glycinamide ribotide transformylase 16.3.3

glycine cleavage system 15.2.6

glycogen

α and β particles 8.2.3

degradation 8.3.58.3.7

lysosomal 8.3.7

metabolism

liver vs. muscle 8.58.5.2

overview 8.18.2.1

regulation 8.48.4.3

structure 8.28.2.4

synthesis 8.3.18.3.4

glycogen phosphorylase 8.3.5

allosteric regulation 8.4.1

liver and muscle isoforms 8.4.3

muscle

deficiency 8.6.3

reaction mechanism 8.3.6

glycogen storage diseases 8.68.6.4

glycogen synthase 8.3.28.3.4

regulation 8.4.1, 13.2.14

glycogen synthase kinase 3 13.2.14

glycogenin 8.3.2

glycolysis 3.13.5.3

anaerobic

ATP yield 3.3.83.4

cell types 3.2

reactions 3.1.23.2

reversible and irreversible reactions 3.2

glyoxylate cycle 10.610.6.1

gout 16.6.216.6.5

dietary factors 16.6.3

guanase 16.5.1, 16.5.3

guano footnote 3

guanosine kinase 16.5.6

guanosine monophsphate (GMP) synthesis 16.3.7

guar gum 11.7.7

Guthrie test 12.5.2

Haber-Bosch process footnote 3

haptocorrin 15.5.4

haptoglobin 11.6.12

HbA1C14.5.8

HDL see high density lipoprotein

Helicobacter pylori 1.6.5

heme

biosynthesis 17.217.2.7

crystallization in malaria parasites 9.4.3

functions 17.1.1

respiratory chain cofactors 6.4.1

heme biosynthesis

disruptions (overview) 17.3

heme degradation 17.417.4.6

heme oxygenase 17.4

hemochromatosis 17.3.4

hemosiderin 17.5.3

heterotrophic

definition 1.4

hexokinase 3.2

and glucokinase 3.5.23.5.3

reaction mechanism 3.33.3.3

hexose monophosphate shunt

homologous sugar series 9.2.3

overview 9.1

reactions 9.29.2.2

hibernating animals 10.3.8

high density lipoprotein 11.4.2, 11.4.8

Hitchhiker’s Guide to the Galaxy 11.6.12

HLA molecules 14.4.114.4.3

homogentisate dioxygenase 12.4.5

hormonal regulation

overview 13.1

hormone-sensitive lipase 10.3.7

Howard Hughes 1.1

human immunodeficiency virus (HIV) 16.9.11

human leukocyte antigens see HLA molecules

hydroxyacyl dehydratase see fatty acid synthase, reactions

hydroxyacyl-CoA dehydrogenase 10.3.3

hydroxybutyrate dehydrogenase 10.4.1

hydroxymethylbilane synthase 17.2.5

hydroxymethylglutaryl-CoA lyase 10.4.1, 12.4.4

hydroxymethylglutaryl-CoA reductase

reaction 11.2.2

regulation 11.3

therapeutic inibition 11.7.2

hydroxymethylglutaryl-CoA synthase

cholesterol synthesis 11.2.2

ketone body synthesis 10.4.1

p-hydroxyphenylpyruvate dioxygenase 12.4.5

therapeutic inhibition in tyrosinemia 12.5.4

hydroxypyruvate 12.4.3

hypercholesterolemia

familial 11.8.1

induction of atherosclerosis 11.611.6.1

hyperlipidemia

glucose-6-phosphatase deficiency 8.6.1

hyperuricemia 16.6.3, see also gout

drug-induced 16.6.5

glucose-6-phosphatase deficiency 8.6.1

Lesch-Nyhan syndrome 16.6.1

hypoglycemia

ethanol-induced 7.4.2

glucose-6-phosphatase deficiency 8.6.1

in vitamin B12 deficiency 7.4.1

hypoxanthine 16.3.4, 16.5.3

hypoxanthine-guanine phosphoribosyltransferase (HGPRT) 16.5.6

deficiency 16.6.1

idoxuridine 16.9.4

inflammasomes 11.6.4

inosine kinase 16.5.6

inosine monophosphate (IMP) 16.3.1, 16.3.7

inosine monophosphate (IMP) dehydrogenase 16.5.1, 16.5.2

inosine monophosphate cyclohydrolase 16.3.4

INSIG 11.3.1

insulin

aggregation 14.5.3

biphasic preparations 14.5.5

discovery 13.2.213.2.3

gluconeogenesis 7.5.37.5.4

glucose transport 13.2.1513.2.16

glycogen metabolism 8.4.2, 13.2.14

mutants, for intensive therapy 14.5.11

primary structure 13.2.513.2.7

protamine complex 14.5.4

secretion

regulation 13.2.813.2.9

secretion, kinetics 14.5.114.5.2

transcriptional regulation 13.2.17

insulin receptor

signaling mechanism 13.2.1213.2.13

intellligent design 6.10.4

intestinal organs see also stomach, liver, etc., 1.6.1

intestine, large 1.6.14

intestine, small

structure 1.6.91.6.10

intrinsic factor 15.5.4

iron depletion 17.3, 17.5

iron overload 17.5.3

iron uptake and storage 17.517.5.3

iron-sulfur clusters 6.4.1

isocitrate 5.4.3

isocitrate dehydrogenase 5.4.3

NAD- and NADP-dependent isoforms 5.5, 6.106.10.4

isocitrate lyase 10.6.1

isomaltose 1.6.12

isoniazid, metabolism 18.3.518.3.6

isopentenyl-CoA 12.4.4

isopentenyl-pyrophosphate isomerase 11.2.3

isouramil 9.4.2

isovaleryl-CoA 12.4.4

isozymes 8.4.3

IUBMB classification of enzymes 2.2.4

jaundice

causes 17.4.1

neonatal 17.4.217.4.5

phototherapy 17.4.4

kernicterus 17.4.3

ketoacidosis 14.3.5

ketoacyl reductase see fatty acid synthase, reactions

ketoacyl synthase see fatty acid synthase, reactions

ketoconazole 18.2.4

ketogenic diet 10.4.4

α-ketoglutarate

citric acid cycle 5.4.3

in transamination 12.2

α-ketoglutarate dehydrogenase 5.4.3

reaction mechanism 5.4.35.4.4

α-ketoisocaproate 12.4.4

ketone body metabolism

dysregulation in diabetes 14.3.4

overview 10.4

reactions 10.4.110.4.3

kidneys

function, overview 14.2.1

glomerular filtration 14.2.4

loss of glucose in diabetes 14.2.6

tissue structure 14.2.214.2.3

tubular reuptake and secretion 14.2.5

lactase see β-galactosidase

lactate 3.2

anaerobic exercise 8.5.3

blood level footnote 13

muscle glycogen utilization 8.5.2

lactate dehydrogenase 3.2, 3.4.13.4.2

lactic acidosis 6.9.2, footnote 85

lactose

degradation 4.34.3.2

intolerance 4.3.24.3.3

structure 4.3

lactose intolerance 4.3.3

Lafora disease 8.6.4

Langerhans islets 13.2.1

lanosterol 11.2.6

LDL see low density lipoprotein

LDL apheresis 11.7.6

lead intoxication 17.2.4, 17.3

lecithin-cholesterol acetyltransferase 11.4.811.4.9

Leloir pathway see galactose degradation

leptin 13.3.8

Lesch-Nyhan syndrome 16.6.1

leucine degradation 12.4.4

linoleate 10.2.1

lipids

types 10.1

lipoamide

in pyruvate dehydrogenase 5.2.3

lipoprotein lipase 10.2.6

lipoproteins

classification 11.4.2

general structure 11.4.1

liver

glycogen content 8.1

glycogen utilization 8.5.1

lobules 1.6.31.6.4

localized enzyme expression 12.3.8

substrate overload in diabetes 14.3.4

liver cirrhosis footnote 73

lomitapide 11.7.3

lovastatin 11.7.2

low density lipoprotein 11.4.2

chemical modifications 11.6.711.6.8

in atherosclerosis 11.6.4

lymphocytes

anaerobic glycolysis 3.2

macrocytic anemia

folate deficiency 15.5.3

in cobalamin deficiency 15.5.6

macrophages

cholesterol accumulation 11.6.611.6.7

conversion to foam cells 11.6.3

production of ROS 9.3.7

malaria 9.4.39.4.4

l-malate 5.4.3

malate dehydrogenase 5.4.3

regulation of the TCA cycle 5.5

malate synthase 10.6.1

malate-aspartate shuttle 6.9.16.9.2

malate-oxaloacetate shuttle 6.96.9.1

maleylacetoacetate isomerase 12.4.5

malonyl-acetyltransferase see fatty acid synthase, reactions

maltose 1.6.12

mannose receptor 19.3.6

mannose-6-phosphate receptor 19.3.419.3.5

Mary Lyon hypothesis 9.4

McArdle’s disease 8.6.3

mechlorethamine 18.5.2

medium-chain fatty acids 10.3.9

mercaptopurine 16.9.6

metabolism

autotrophic and heterotrophic 1.4

carbon pools 10.1.2

catabolic and anabolic 1.3

drug targets in 1.2

hormonal regulation, overview 13.1

in plants and bacteria 1.4

medical significance 1.2

metformin 14.5.1414.5.15

methemoglobin reductase 17.1.1, 18.4, 18.5.3

methotrexate 16.9.5

methyl trap hypothesis 15.5.6

methylcobalamin see cobalamin

methylglutaconyl-CoA 12.4.4

methylmalonyl-CoA racemase 10.3.6

mevalonate kinase 11.2.2

mevastatin 11.7.2

mifepristone 13.3.7

miglustat 19.3.8

minimal medium 12.5.2

mitochondria

hydrogen shuttles 6.8.56.9.3

inner and outer membrane 5.1.1

proton leak 6.10, footnote 36

proton-motive force 6.86.8.1

transport driven by 6.11.16.11.2

mitochondrial triglyceride transfer protein 11.4.3, 11.7.3

mixed acid fermentation 4.3.3

morphine

metabolism 18.3.4

muscle see also skeletal muscle

smooth vs. striated 3.1

muscle tissue

fatty acid utilization 10.3.7

myelin 15.4.7

myeloperoxidase 9.3.7, 11.6.12

N,N’-methylene-tetrahydrofolate see also folic acid

regeneration 15.2.415.2.7

N-acetylglutamate 12.3.9

N-acetyltransferase 2 18.3.518.3.6

NAD

and NADP 3.3.6

comparison 9.3.1

transhydrogenase 6.10.16.10.4

in β-oxidation 10.3.3

mitochondrial dehydrogenation of NADH 6.66.6.1, 14.5.14

redox mechanism 3.3.53.3.6

reoxidation of NADH under anaerobic conditions 3.43.4.3

NADPH

metabolic uses, overview 9.3.4

oxidase 9.3.7

regeneration

hexose monophosphate shunt 9.1, 9.29.2.1

malic enzyme 9.3.2

transhydrogenase 9.3.3

neonatal jaundice 17.4.217.4.5

nicotinamide adenine dinucleotide see NAD, NADH

nicotinamide adenine dinucleotide phosphate see NADP, NADPH

nicotinamide nucleotide transhydrogenase 6.10.16.10.4

nicotinic acid 11.7.7

nitric oxide

signaling effects 9.3.6, 17.4.6

synthase 9.3.5

2-[2-nitro-4-(trifluoromethyl)benzoyl]cyclohexane-1,3-dione 12.4.5, 12.5.4

nitrogen disposal

overview 12.3

transport between organs 12.3.512.3.6

nitrogen fixation 1.4

NPC1L1 11.4.3, 11.7.3

NTBC 12.4.5, 12.5.4

nuclear hormone receptors 13.3.413.3.5

nucleic acids

dietary, utilization 16.416.4.2

5′-nucleotidase 16.9.816.9.9

nucleotide antimetabolites 16.9

nucleotide synthesis see purine synthesis, pyrimidine synthesis

nucleotides

as part of coenzymes 16.1.2

biochemical roles 16.1.1

dietary, utilization 16.416.4.2

pathways

overview 16.216.2.1

ochratoxin A 12.5.3

oleate 10.2.1

organic anion transport proteins 11.5.3

ornithine aminotransferase 12.3.8

ornithine transcarbamylase 12.3.2

orotate phosphoribosyltransferase 16.7.1

orotidine monophosphate (OMP) 16.7.1

osmotic diuresis 14.2.6

oxaloacetate 5.4.3

malate-aspartate shuttle 6.9.2

oxalosuccinate 5.4.3

oxidized LDL 11.6.811.6.9

role in atherosclerosis 11.6.12

P-glycoprotein 16.9.8

palmitate 10.2.1

fatty acid synthesis 10.5

pancreas

endocrine 13.2.1

exocrine 1.6.7

insufficiency 10.3.9

pancreatic DNAse and RNAse 16.4.1

pancreatic lipase 10.2.2

parietal cells 15.5.5

pentose posphate shunt see hexose monophosphate shunt

pentostatin 19.2.2

PEP see phosphoenolpyruvate

pernicious anemia 15.5.5

phagocytes

production of ROS 9.3.7

phenobarbital, metabolism 18.1.2

phenylacetic acid 18.3.7

phenylalanine degradation 12.4.5

phenylalanine hydroxylase 12.4.5

deficiency 12.5.1

phenylbutanoic acid 18.3.7

phenylketonuria 12.5.112.5.3

phosphatidylcholine

biosynthesis 15.4.5

LCAT reaction 11.4.8

sphingomyelin biosynthesis 15.4.6

phosphatidylethanolamine 15.4.5

phosphatidylinositol-3-kinase 13.2.14

phosphodiesterase 7.5.4, 13.2.14

phosphoenolpyruvate 3.2, 3.3.7, 3.3.8

phosphoenolpyruvate carboxykinase 7.2.47.2.5

phosphofructokinase 3.2

allosteric regulation 2.5.12.5.3, 7.5.3

phosphofructokinase 2 7.5.4

phosphoglucomutase

galactose degradation 4.3.1

glycogen degradation 8.3.5

glycogen synthesis 8.3.1

6-phosphogluconate 9.2.1

6-phosphogluconolactone 9.2.1

phosphoglycerate kinase 3.2

phosphoglycerate mutase 3.2

2-phosphoglycerate 3.2

3-phosphoglycerate 3.2

phosphohexose isomerase 3.2

phosphohexoseisomerase

reaction mechanism 3.3.33.3.4

phosphomevalonate kinase 11.2.2

phosphopantetheine 10.5.3

phosphopentomutase 16.4.2

5′-phosphoribosyl-1′-pyrophosphate 16.3.1, 16.7.1

phosphoribosylamine 16.3.2, 16.3.8

5′-posphoribosyl-1′-pyrophosphate 16.3.2

phosphorylase see glycogen phosphorylase

photosynthesis 1.4

ping pong bi bi enzyme reaction mechanism 12.2.2

plant sterols 11.4.4, 11.7.3

polyol pathway 4.3.44.4

in diabetic polyneuropathy 4.4

Pompe disease 8.6.2

enzyme therapy 19.3.219.3.3

porphobilinogen deaminase 17.2.5

deficiency 17.3.5

porphobilinogen synthase 17.2.317.2.4

porphyria cutanea tarda 17.3.217.3.4

prednisolone 13.3.7

pregnane X receptor 18.2.2

priapism 19.2.3

primaquine 9.4.4

probenecid 16.6.5

probucol 11.7.7

propionate

in β-oxidation 10.3.6

propionyl-CoA carboxylase 10.3.6

proteasome 2.2.1

proteasomes 2.5.8

protein kinase A 7.5.4, 8.4.2, 10.3.7, 13.3.3

protein kinase B 13.2.14

protoporphyrinogen IX 17.2.7

PRPP see 5′-phosphoribosyl-1′-pyrophosphate

purine nucleoside phosphorylase 16.4.1, 16.5.2

purine nucleotide cycle 16.3.6

purine nucleotide degradation 16.5.116.5.5

purine nucleotide salvage 16.5.6

purine nucleotide synthesis

overview 16.316.3.1

reactions 16.3.216.3.7

regulation 16.3.8

pyrazinamide, metabolites 16.6.5

pyridoxal phosphate

amino acid transamination 12.2.1

pyridoxal phosphate

δ-aminolevulinate synthase 17.2.2

glycogen phosphorylase 8.3.6

serine dehydratase 12.4.2

serine hydroxymethyltransferase 15.2.4

pyrimidine nucleoside phosphorylase 16.4.1, 16.7.3

pyrimidine nucleotide degradation 16.7.3

pyrimidine nucleotide synthesis 16.7.116.7.2

pyrophosphatase footnote 43

pyruvate

mitochondrial transport 5.1.1

pyruvate carboxylase

and citric acid cycle 7.4

mechanism 7.27.2.4

role in citric acid cycle 5.6

pyruvate dehydrogenase 5.1.15.3.2

coenyzmes 5.2.35.2.4

kinase 5.3.2

phosphatase 5.3.2

reaction 5.2.1

reaction mechanisms 5.2.35.2.7

regulation 5.35.3.2

subunit structure 5.2.15.2.3

pyruvate kinase 3.2

reaction mechanism 3.3.63.3.7

regulation 7.5.6

Q cycle 6.6.16.6.3

Racker experiment 6.3.26.3.3

rasburicase 16.6.7

reactive oxygen species

formation by hemoglobin 17.1.1

formation in phagocytes 9.3.7

formation in the respiratory chain 6.4.3

lipid peroxidation 11.6.1011.6.11

NADPH and glutathione in scavenging 9.3.8

receptor tyrosine kinases 13.2.12

red blood cells

anaerobic glycolysis 3.2

redox potential

and free energy 6.5.16.5.2

measurement 6.56.5.1

respiratory acidosis footnote 85

respiratory chain

overview 6.16.2.1

protein complexes 6.3.36.4

proton pumping

mechanism 6.6.46.7.1

stoichiometry 6.7.16.7.2

redox cofactors 6.46.4.2

hydrogen and electron carriers 6.6

single- and two-electron carriers 6.6

redox potentials and free energies 6.5.26.5.3

regulation 6.106.10.4

uncoupling proteins 6.36.3.1

retinoid X receptor 13.3.5

ribonucleotide reductase 16.8

ribose phosphate diphosphokinase 16.3.2

ribose-5-phosphate

hexose monophosphate shunt 9.2.2

ribosephosphate diphosphokinase 16.9.6

ribulose-5-phosphate 9.2.1

epimerase 9.2.2

isomerase 9.2.2

rickets 11.2.7

RNA world 16.1.316.1.4

Rolls Royce 1.1

S-adenosylhomocysteine 15.4.3

S-adenosylmethionine

cycle 15.4.2

role in biosynthesis 15.4.415.4.5

structure 15.4.3

Saccharomyces cerevisiae 3.4.3

SAICAR see purine nucleotide synthesis

salicylic acid 16.6.5

Sanger, Frederick 13.2.5

SCAP 11.3.111.3.3

scavenger receptor 11.6.6

sedoheptulose-7-phosphate 9.2.2

serine dehydratase 12.4.2

serine hydroxymethyltransferase 15.2.4

serine-pyruvate transaminase 12.4.3

serotonin, deficit in phenylketonuria 12.5.1

severe combined immunodeficiency 19.2

Sherlock Holmes 1.1

Sinorhizobium meliloti 1.4

sitosterol 11.7.3

structure 11.4.4

sitosterolemia 11.8.3

skeletal muscle

Cori cycle 8.5.3

glycogen content 8.1

glycogen utilization 8.5.2

skin pigmentation 11.2.7

slow acetylators 18.3.6

smooth muscle footnote 11

Sn-mesoporphyrin 17.4.5

sodium glucose transporter (SGLT1) 1.6.13

sorbitol 4.4

sphingomyelin 15.4.6

squalene

cyclization 11.2.5

synthesis 11.2.4

SREBP 11.3.111.3.3

SREBP cleavage activating protein (SCAP) 11.3.111.3.3

standard hydrogen electrode 6.5.1

standard redox potential 6.5.1

statins 11.7.2

stearate 10.2.1

sterol response element-binding protein (SREBP) 11.3.111.3.3

stomach 1.6.51.6.6

parietal cells 15.5.5

striated muscle footnote 11

substrate cycles

in gluconeogenesis 7.57.5.3

metabolic regulation 2.5.7

succinate 5.4.3

succinate dehydrogenase 5.4.3

succinate thiokinase 5.4.3

succinobucol 11.7.7

succinyl-aminoimidazole-carboxamido-ribotide synthetase 16.3.3

succinyl-CoA

propionate utilization 10.3.6

TCA cycle 5.4.3

succinyl-CoA mutase 10.3.6

succinylphosphate 3.3.8

sucrase 4.2

sucrose

degradation 4.24.2.1

sugar beet 4.2

sugar cane 4.2

sulfonamides 15.3

sulfonylurea receptor 13.2.813.2.11

superoxide see reactive oxygen species

superoxide dismutase 9.3.7

T cell receptors 14.4.1

T lymphocytes, subtypes and functions 14.4.114.4.2

Tangier disease 11.8.2

TCA cycle see citric acid cycle

tenofovir disoproxil 16.9.14

terminal ileum

bile acid reuptake 11.5.2

cobalamin uptake 15.5.4

tetrahydrofolate

role in biosynthesis 15.2.1, 15.2.3, 15.2.9

thermogenin 10.3.8

thiamine pyrophosphate

pyruvate dehydrogenase 5.2.45.2.6

transketolase 9.2.4

thioester

as an energy-rich group 3.3.8

in the glyceraldehyde-3-phosphate dehydrogenase reaction 3.3.5

thioguanine 16.9.6

thiolase

cholesterol synthesis 11.2.2

reaction mechanism 10.3.5

thiopurine methyltransferase 16.9.6

thioredoxin 18.4

thrombocytes

anaerobic glycolysis 3.2

thymidylate synthase 16.8.1, 16.9.2

thyroid hormones

signaling mechanims 13.3.413.3.5

tocopherol 11.6.11

tolbutamide 13.2.11

transaldolase 9.2.2

reaction mechanism 9.2.5

transamination see amino acids, transamination

branched chain amino acids 12.4.4

transcobalamin 15.5.4

transition state 2.3, 2.3.3

transketolase 9.2.2

IUBMB nomenclature 2.2.4

reaction mechanism 9.2.4

translational antitermination 19.1.319.1.4

triacylglycerol

digestion and uptake 10.210.2.3

efficiency of energy storage 10.1.1

solubilization by bile acids 10.2.2

structure 10.2.1

utilization, organ relationships 10.3.7

triacylglycerol lipase 10.2.2

trimethoprim 15.3

triose phosphate isomerase 3.2

triparanol 11.7.7

tumor lysis syndrome 16.6.616.6.7

tyrosine degradation 12.4.5

tyrosine transaminase 12.4.5

tyrosinemia 12.5.4

ubiquinone see coenzyme Q, 6.4.2

ubiquitin 2.5.8

UDP-galactose 4.3.1

UDP-glucose

in galactose degradation 4.3.1

UDP-galactose epimerase 4.3.1, 4.3.4

UDP-glucose

glycogen synthesis 8.3.1

UDP-glucuronic acid

biosynthesis 8.3.1

ulcers, gastric and duodenal 1.6.5

uncoupling proteins 6.36.3.1, 13.3.5

URAT1 transporter 16.5.4

urate oxidase see uricase

urea cycle

distribution of enzyme activity 12.3.8

enzyme defects 12.3.10

alternate pathway therapy 18.3.618.3.7

integration with other pathways 12.3.312.3.6

reactions 12.3.112.3.2

regulation 12.3.9

uric acid

degradation in non-primates 16.5.5

formation 16.5.3

renal elimination 16.5.4

uricase 16.5.5

therapeutic use 16.6.7

uricosuric drugs 16.5.4, 16.6.5

uridine triphosphate (UTP)

glycogen synthesis 8.3.1

uroporphyrinogen decarboxylase 17.2.6

uroporphyrinogen III cosynthase 17.2.6

uroporphyrinogen III decarboxylase

deficiency 17.3.2

very low density lipoprotein 11.4.2

vitamin B12see cobalamin

propionate utilization 10.3.6

vitamin B6 deficiency 17.3

vitamin D 11.2.7

vitamin E 11.6.11

VLDL see very low density lipoprotein

von Gierke disease 8.6.1

xanthine 16.5.3

xanthine dehydrogenase/oxidase 16.5.116.5.3, 16.6.5, 16.9.6

xanthosine monophosphate (XMP) 16.3.7

xenobiotic response elements 18.2.2

xylose 3.3.3

xylulose-5-phosphate 9.2.2