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 11.4.5–11.4.6
ACAT see acyl-CoA cholesterol acyltransferase
acetaminophen, metabolism 19.3.3
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
substrate for gluconeogenesis 10.4.3
acetyl-CoA
formation by pyruvate dehydrogenase 5.2.1, 5.2.7
mitochondrial export 10.5.6–10.5.7
oxidation see citric acid cycle
acetyl-CoA carboxylase 10.5.1
acetylphosphate 3.3.8
acid maltase 8.3.7
deficiency 8.6.2
acid-base catalysis 3.3.3–3.3.4
activation energy (ΔG*) 2.3
active transport
glucose 1.6.13
acute intermittent porphyria 17.3.6
acute myeloic leukemia (AML) 16.9.9
acute urate nephropathy 16.6.6–16.6.7
acyl carrier protein footnote 66
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 20.2.1
experimental drug treatment 20.2.2
gene therapy 20.2.5
pathogenesis 20.2
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 2.5.1–2.5.2, 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
deficiency (fructose intolerance) 4.2.2
aldose reductase 4.4
inhibitors, in diabetes 14.5.7
allantoin 16.5.5
amino acids
conjugation 19.3.8
degradation
asparagine 12.4.1
glycine 15.2.6
histidine 15.2.7
leucine 12.4.4
phenylalanine, tyrosine 12.4.5
serine 12.4.2–12.4.3, 15.2.4–15.2.5
metabolic uses 12.1
transamination
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 79
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 19.5.1, 20.2, footnote 35
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
arylamines
metabolic activation as carcinogens 19.3.7
regeneration 18.7.14
asparaginase 12.4.1
asparagine synthetase 12.4.1
aspartate aminotransferase 6.9.2, 12.3.3
aspartate transcarbamylase 16.7.1
atherosclerosis
and hypercholesterolemia 11.6–11.6.1
blood clot formation 11.6.2
metabolic aspects 11.6.5
role of oxidized LDL 11.6.10
vascular lesions
pathogenesis 11.6.4
atorvastatin 11.7.2
ATP
hydrolysis 3.3.1
role in enzyme reactions 2.4–2.4.1
ATP synthase
binding-change model 6.8.2–6.8.3
driven by proton flux 6.8.3–6.8.5
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 10.3.4–10.3.5
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 11.5.2–11.5.3
structure 11.5.1
bilirubin 18.1
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
C-peptide 13.2.6
canfosfamide 19.5.4
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 19.5.3
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 11.7–11.7.1
synthesis
overview 11.2.1
transport by lipoproteins 11.4.7–11.4.9
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 10.2.3–10.2.5
vs. other lipoproteins 11.4.2
chymotrypsin
cidofovir 16.9.14
citrate isomerase 5.4.3
mitochondrial acetyl-CoA export 10.5.6
overall reaction 5.4.1
regulation 5.5
replenishment of intermediates 5.5–5.6
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 ubiquinone, see also Q cycle
coenzymes
role in catalysis 2.2.3
coproporphyrinogen III 17.2.6
Cori cycle 8.5.3
covalent catalysis 3.3.4–3.3.5
coxsackievirus, and type 1 diabetes 14.4
Crigler-Najjar syndrome 17.4.2
Crohn’s disease 15.5.5
cyanide poisoning footnote 102
cobalamin treatment 15.5.5
cyclic AMP (cAMP)
dysregulation in diabetes mellitus 14.3.1
regulation of gluconeogenesis 7.5.3–7.5.5
regulation of glycogen metabolism 8.4.2
cyclic GMP (cGMP) 9.3.6
cyclooxygenase
mechanism 18.5.9
products 18.5.7
cystic fibrosis 20.1.4
cytarabine see cytosine arabinoside
cytochrome C 6.4, 6.4.2, 6.6.3
cytochrome C oxidase 6.4
reaction mechanism 6.6.3–6.6.4
cytochrome P450
acetone metabolism 10.4.3
in drug metabolism 19.2–19.2.6
prominent role of CYP3A4 19.2.2
structure 19.2.3
toxification of benzopyrene 19.2.7
transcriptional induction 19.2.2
cytochrome P450 reductase 19.4
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 20.2.2
deoxycytidine kinase 20.2.2
deoxyribonucleotide synthesis 16.8–16.8.1
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 88
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 14.3.2–14.3.4
etymology 14.1.1
forms 14.1.2
hyperglycemia 14.5.6
hypoglycemia 14.5.6
insulin therapy, intensive 14.5.9–14.5.11
laboratory findings 14.3.5
oral antidiabetics 14.5.12–14.5.13
renal glucose loss 14.2.6
therapy
acute case 14.5.1
type 1, pathogenesis 14.4–14.4.3
dideoxyadenosine 16.9.11
digestion
bile, role of 1.6.8
enzymes 1.6.7
overview 1.5.1
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
diphosphomevalonate decarboxylase 11.2.2
DNA polymerase, inhibitors 16.9.10–16.9.14
DNA topoisomerase 16.9.10
drug metabolism
active metabolites 19.2.6
conjugation reactions 19.3–19.3.8
phase I, II, and III 19.1.3
reductive 19.4
Dubin-Johnson syndrome 11.5.4
earthquakes 11.2.7
eliglustat 20.3.8
elongases 10.5.8
endoplasmic reticulum
smooth
sterol metabolism 11.2.8
energy-rich bonds and functional groups 3.3.7–3.3.8
enolase 3.2
enoyl-CoA hydratase 10.3.3
enoylreductase see fatty acid synthase, reactions
enzyme activity
allosteric regulation 2.5.4
control by phosphorylation 2.5.5
transcriptional control 2.5.7–2.5.8
enzyme defects see also specific enzymes
enzyme therapy
adenosine deaminase deficiency 20.2.3–20.2.4, 20.3.6–20.3.7
gene therapy
adenosine deaminase deficiency 20.2.5
technical considerations 20.1.5
lysosomal, enzyme therapy 20.3–20.3.7
therapeutic options 20.1.1–20.1.2
enzymes, IUBMB classification 2.2.4
epinephrine
glycogen metabolism 8.4.2
hormone-sensitive lipase 10.3.7
signaling mechanism 13.3.1–13.3.3
epoxide hydrolase 19.3.2
equilibrative nucleoside transporter (ENT) 16.9.8
erythromycin 19.2.3
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 19.3.6
fat tissue
white and brown 10.3.8
fatty acid synthase
pharmacological inhibition 10.5.9–10.5.10
structure and catalytic activities 10.5.2–10.5.3
fatty acids
common variants 10.2.1
elongation and desaturation 10.5.8
essential 10.5.8, footnote 56
mitochondrial transport 10.3.1–10.3.2
synthesis
overview 10.5
odd-numbered chain lengths 10.3.6
Fenton reaction 18.2.5
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
foam cells 11.6.3
folic acid
antimetabolites
antimicrobials 15.3
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
fructolysis see fructose degradation
fructose
and obesity 4.2.1
hyperuricemia 16.6.4
in sperm fluid 4.4
fructose-1-phosphate 4.2.1
fructose-1,6-bisphosphatase 7.2.5–7.2.6
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 7.5.3–7.5.5
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
galactitol 4.4
galactose
enzyme defects see galactosemia
galactose epimerase
reaction mechanism 4.3.1–4.3.2
galactose-1-phosphate 4.3.1, 4.3.4
galactose-1-phosphate uridyltransferase 4.3.1, 4.3.4
γ-aminobutyrate (GABA) 17.3.6
ganciclovir 16.9.12
gastric acid 1.6.5
drug treatment 20.3.8
geranyl-pyrophosphate synthase 11.2.3
Gilbert’s syndrome 17.4.2
glucacon
signaling mechanism 13.3.1–13.3.3
glucagon 13.2.1
glycogen metabolism 8.4.2
hormone-sensitive lipase 10.3.7
glucocerebrosidase 20.3.6–20.3.7
partial deglycosylation for enzyme therapy 20.3.7
glucocorticoids
metabolic effects 13.3.6
receptor agonists and antagonists 13.3.7
signaling mechanism 13.3.4–13.3.5
gluconeogenesis
inhibition by ethanol 7.4.2
mitochondrial transport in 7.4.1
gluconolactonase 9.2.1
glucose
active transport 1.6.13
anomers 3.1.2
degradation
GLUT transporters 1.6.13
regulation by insulin 13.2.16
subtypes and kinetics 3.5–3.5.2
intestinal uptake 1.6.13
organ-specific utilization 3.5–3.5.3
glucose alanine cycle 12.3.5
glucose-1-phosphate 4.3.1, 8.3.5
glucose-6-phosphatase 7.2.5–7.2.6
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
neonatal jaundice 17.4.5
reaction 9.2.1
glucosylceramide 20.3.6
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
regulation 12.3.9
glutamine
as a nitrogen carrier 12.3.6
glutamine synthetase 2.4.1, 12.3.7–12.3.8
glutamine-PRPP-amidotransferase 16.3.2
glutaredoxins 18.7.5
glutathione
intracellular concentration 18.1
peroxidase 9.4.2
scavenging of reactive oxygen species 9.3.8
glutathione peroxidase 18.7.5, 18.7.7
glutathione-S-transferase 19.3.2
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 35
reaction mechanism 3.3.4–3.3.5
glycerol 4.2.1
glycerol-1-phosphate 4.2.1
glycerophosphate shuttle 6.9.2–6.9.3
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
lysosomal 8.3.7
metabolism
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 8.6–8.6.4
glycogen synthase kinase 3 13.2.14
glycogenin 8.3.2
anaerobic
cell types 3.2
reversible and irreversible reactions 3.2
dietary factors 16.6.3
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
Haber-Weiss reaction 18.2.5
haptocorrin 15.5.4
haptoglobin 11.6.10
HbA1C14.5.8
HDL see high density lipoprotein
Helicobacter pylori 1.6.5
heme
crystallization in malaria parasites 9.4.3
functions 17.1.1
respiratory chain cofactors 6.4.1
heme biosynthesis
disruptions (overview) 17.3
heme oxygenase 17.4
hemochromatosis 17.3.4
hemosiderin 17.5.3
heterotrophic
definition 1.4
hexokinase 3.2
hexose monophosphate shunt
homologous sugar series 9.2.3
overview 9.1
hibernating animals 10.3.8
high density lipoprotein 11.4.2, 11.4.8
Hitchhiker’s Guide to the Galaxy 11.6.10
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
hydroxyl radical
formation by ionizing radiation 18.2–18.2.2
formation by transition metals 18.2.5
reactions with DNA 18.4–18.4.2
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
reaction with DNA 18.5.5
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 11.6–11.6.1
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-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
glucose transport 13.2.15–13.2.16
glycogen metabolism 8.4.2, 13.2.14
mutants, for intensive therapy 14.5.11
primary structure 13.2.5–13.2.7
protamine complex 14.5.4
secretion
secretion, kinetics 14.5.1–14.5.2
transcriptional regulation 13.2.17
insulin receptor
signaling mechanism 13.2.12–13.2.13
intellligent design 6.10.4
intestinal organs see also stomach, liver, etc., 1.6.1
intestine, large 1.6.14
intestine, small
intrinsic factor 15.5.4
iron overload 17.5.3
iron uptake and storage 17.5–17.5.3
iron-sulfur clusters 6.4.1
isocitrate 5.4.3
isocitrate dehydrogenase 5.4.3
NAD- and NADP-dependent isoforms 5.5, 6.10–6.10.4
isocitrate lyase 10.6.1
isomaltose 1.6.12
isoniazid, metabolism 19.3.5–19.3.6
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
kernicterus 17.4.3
ketoacidosis 14.3.5
ketoacyl reductase see fatty acid synthase, reactions
ketoacyl synthase see fatty acid synthase, reactions
ketoconazole 19.2.4
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 5.4.3–5.4.4
α-ketoisocaproate 12.4.4
ketone body metabolism
dysregulation in diabetes 14.3.4
overview 10.4
kidneys
function, overview 14.2.1
glomerular filtration 14.2.4
loss of glucose in diabetes 14.2.6
tissue structure 14.2.2–14.2.3
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, 3.4.1–3.4.2
lactic acidosis 6.9.2, footnote 91
lactose
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 11.4.8–11.4.9
Leloir pathway see galactose degradation
leptin 13.3.8
Lesch-Nyhan syndrome 16.6.1
leucine degradation 12.4.4
leukotrienes 18.5.7
linoleate 10.2.1
lipid peroxidation 18.4.3–18.5.6
and DNA modification 18.5.5
by cyclooxygenase and lipoxygenase 18.5.7
inhibition by tocopherol 18.7.11
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
lipoxygenase
mechanism 18.5.8
products 18.5.7
liver
glycogen content 8.1
glycogen utilization 8.5.1
localized enzyme expression 12.3.8
substrate overload in diabetes 14.3.4
liver cirrhosis footnote 79
lomitapide 11.7.3
lovastatin 11.7.2
low density lipoprotein 11.4.2
chemical modifications 11.6.7–11.6.8
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 11.6.6–11.6.7
conversion to foam cells 11.6.3
production of ROS 9.3.7
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 6.9.1–6.9.2
malate-oxaloacetate shuttle 6.9–6.9.1
maleylacetoacetate isomerase 12.4.5
detection with thiobarbituric acid 18.5.6
reaction with DNA 18.5.5
malonyl-acetyltransferase see fatty acid synthase, reactions
maltose 1.6.12
mannose receptor 20.3.6
mannose-6-phosphate receptor 20.3.4–20.3.5
Mary Lyon hypothesis 9.4
McArdle’s disease 8.6.3
mechlorethamine 19.5.2
medium-chain fatty acids 10.2.7
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
metallothioneins 18.7.2
methemoglobin reductase 17.1.1, 19.4, 19.5.3
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 20.3.8
minimal medium 12.5.2
mitochondria
inner and outer membrane 5.1.1
proton leak 6.10, footnote 39
transport driven by 6.11.1–6.11.2
mitochondrial triglyceride transfer protein 11.4.3, 11.7.3
mixed acid fermentation 4.3.3
morphine
metabolism 19.3.4
muscle see also skeletal muscle
smooth vs. striated 3.1
muscle tissue
fatty acid utilization 10.3.7
myelin 15.4.7
N,N’-methylene-tetrahydrofolate see also folic acid
N-acetylglutamate 12.3.9
N-acetyltransferase 2 19.3.5–19.3.6
NAD
and NADP 3.3.6
comparison 9.3.1
transhydrogenase 6.10.1–6.10.4
in β-oxidation 10.3.3
mitochondrial dehydrogenation of NADH 6.6–6.6.1, 14.5.14
reoxidation of NADH under anaerobic conditions 3.4–3.4.3
NADPH
metabolic uses, overview 9.3.4
oxidase 9.3.7
regeneration
hexose monophosphate shunt 9.1, 9.2–9.2.1
malic enzyme 9.3.2
transhydrogenase 9.3.3
neonatal jaundice 17.4.2–17.4.5
nicotinamide adenine dinucleotide see NAD, NADH
nicotinamide adenine dinucleotide phosphate see NADP, NADPH
nicotinamide nucleotide transhydrogenase 6.10.1–6.10.4
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 12.3.5–12.3.6
nitrogen fixation 1.4
nuclear hormone receptors 13.3.4–13.3.5
nucleic acids
dietary, utilization 16.4–16.4.2
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 16.4–16.4.2
pathways
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
role in atherosclerosis 11.6.10
P-glycoprotein 16.9.8
palmitate 10.2.1
fatty acid synthesis 10.5
pancreas
endocrine 13.2.1
exocrine 1.6.7
insufficiency 10.2.7
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 20.2.2
PEP see phosphoenolpyruvate
pernicious anemia 15.5.5
phagocytes
production of ROS 9.3.7
phenobarbital, metabolism 19.1.2
phenylacetic acid 19.3.8
phenylalanine degradation 12.4.5
phenylalanine hydroxylase 12.4.5
deficiency 12.5.1
phenylbutanoic acid 19.3.8
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 7.2.4–7.2.5
phosphofructokinase 3.2
allosteric regulation 2.5.1–2.5.3, 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 3.3.3–3.3.4
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
in diabetic polyneuropathy 4.4
Pompe disease 8.6.2
porphobilinogen deaminase 17.2.5
deficiency 17.3.6
porphobilinogen synthase 17.2.3–17.2.4
porphyria cutanea tarda 17.3.2–17.3.4
prednisolone 13.3.7
pregnane X receptor 19.2.2
priapism 20.2.3
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
prostaglandins 18.5.7
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
protein tyrosine phosphatase Cdc25B 18.2.9
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 16.5.1–16.5.5
purine nucleotide salvage 16.5.6
purine nucleotide synthesis
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 16.7.1–16.7.2
pyrophosphatase footnote 47
pyruvate
mitochondrial transport 5.1.1
pyruvate carboxylase
and citric acid cycle 7.4
role in citric acid cycle 5.6
pyruvate dehydrogenase 5.1.1–5.3.2
kinase 5.3.2
phosphatase 5.3.2
reaction 5.2.1
reaction mechanisms 5.2.3–5.2.7
pyruvate kinase 3.2
reaction mechanism 3.3.6–3.3.7
regulation 7.5.6
radical reactions 18.2.2–18.2.6
with macromolecules 18.3.5–18.4.3
rasburicase 16.6.7
reactive oxygen species
formation by hemoglobin 17.1.1
formation in phagocytes 9.3.7, 18.3–18.3.3
formation in the respiratory chain 6.4.3, 18.3.3–18.3.5
NADPH and glutathione in scavenging 9.3.8
reactive species
from ionizing radiation 18.2–18.2.2
measurment of reactivity 18.2.6–18.2.8
protective mechanisms 18.6.4–18.7.14
protein modification 18.4.3
radical reactions 18.2.2–18.2.6
receptor tyrosine kinases 13.2.12
red blood cells
anaerobic glycolysis 3.2
redox potential
respiratory acidosis footnote 91
respiratory chain
proton pumping
hydrogen and electron carriers 6.6
single- and two-electron carriers 6.6
redox potentials and free energies 6.5.2–6.5.3
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
Rolls Royce 1.1
S-adenosylhomocysteine 15.4.2
S-adenosylmethionine
cycle 15.4.2
role in biosynthesis 15.4.4–15.4.5
structure 15.4.3
Saccharomyces cerevisiae 3.4.3
SAICAR see purine nucleotide synthesis
salicylic acid 16.6.5
Sanger, Frederick 13.2.5
scavenger receptor 11.6.6
sedoheptulose-7-phosphate 9.2.2
selenoprotein P 18.7.12
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 20.2
Sherlock Holmes 1.1
and lipid peroxidation 18.6.2–18.6.4
and porphyria 18.6.3
formation by porphyrins 18.6.1
reactivity 18.6.2
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 19.3.6
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 cleavage activating protein (SCAP) 11.3.1–11.3.3
standard hydrogen electrode 6.5.1
standard redox potential 6.5.1
of selected radicals 18.2.9
statins 11.7.2
stearate 10.2.1
sterol response element-binding protein (SREBP) 11.3.1–11.3.3
parietal cells 15.5.5
striated muscle footnote 11
substrate cycles
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
sugar beet 4.2
sugar cane 4.2
sulfonamides 15.3
sulfonylurea receptor 13.2.8–13.2.11
superoxide 6.4.3
superoxide dismutase 9.3.7, 18.7.2–18.7.3
T cell receptors 14.4.1
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 5.2.4–5.2.6
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 reductase 18.7.6
thrombocytes
anaerobic glycolysis 3.2
thymidylate synthase 16.8.1, 16.9.2
thyroid hormones
signaling mechanims 13.3.4–13.3.5
thyroid peroxidase 18.1.1
tocopherol
antioxidant function 18.7.11
regeneration by ubiquinol 18.7.13
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 metals
formation of hydroxyl radicals 18.2.5
transketolase 9.2.2
IUBMB nomenclature 2.2.4
reaction mechanism 9.2.4
translational antitermination 20.1.3–20.1.4
triacylglycerol
digestion and uptake 10.2–10.2.3
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 16.6.6–16.6.7
tyrosine degradation 12.4.5
tyrosine transaminase 12.4.5
tyrosinemia 12.5.4
antioxidant function 18.7.12–18.7.14
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 6.3–6.3.1, 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 19.3.7–19.3.8
integration with other pathways 12.3.3–12.3.6
regulation 12.3.9
uric acid
antioxidant function 18.7.10
regeneration 18.7.14
degradation in non-primates 16.5.5
formation 16.5.3
renal elimination 16.5.4
renal elimination and reuptake 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 18.7.11
VLDL see very low density lipoprotein
von Gierke disease 8.6.1
xanthine 16.5.3
xanthine dehydrogenase/oxidase 16.5.1–16.5.3, 16.6.5, 16.9.6
xanthosine monophosphate (XMP) 16.3.7
xenobiotic response elements 19.2.2
xylose 3.3.3
xylulose-5-phosphate 9.2.2