click below
click below
Normal Size Small Size show me how
Med Biochem Enzymes
Medical biochemistry enzymes
| Enzyme | Reaction/Fxn | Pathway |
|---|---|---|
| Hexokinase | Glucose --> G-6-P | Glycolysis |
| Phosphoglucoisomerase | G-6-P <---> F-6-P | Glycolysis/Gluconeogenesis |
| Phosphofructokinase (PFK) | F-6-P --> F-1,6-bisP | Glycolysis |
| Aldolase | F-1,6-bisP <----> Glyceraldehyde 3-phosphate & Dihydroxyacetone Phosphate | Glycolysis/Gluconeogenesis |
| Triose Phosphate Isomerase | Dihydroxyacetone Phosphate <----> Glyceraldehyde 3-phosphate | Glycolysis/Gluconeogenesis |
| Glyceraldehyde 3-phosphate Dehydrogenase | Glyceraldehyde 3-phosphate <---> 1,3 Bisphosphoglycerate | Glycolysis/Gluconeogenesis |
| Phosphoglycerate Kinase | 1,3 Bisphosphogycerate <---> 3-phosphoglycerate | Glycolysis/Gluconeogenesis |
| Pyruvate Kinase | Phosphoenolpyruvate(PEP) --> Pyruvate | Glycolysis |
| Pyruvate Carboxylase | Pyruvate --> Oxaloacetate | Gluconeogenesis |
| Phosphoenolpyruvate Carboxykinase (PEPCK) | Oxaloacetate --> Phosphoenolpyruvate (PEP) | Gluconeogenesis |
| Fructose 1,6-bisphosphatase | F-1,6-bisP --> F-6-P | Gluconeogenesis |
| Glucose 6-phosphatases | G-6-P --> Glucose | Gluconeogenesis |
| Pyruvate Dehydrogenase (E1) | TPP + Pyruvate --> Hydroxyethyl-TPP | Pyruvate Dehydrogenase Complex |
| Dihydrolipoyl Transacetylase (E2 | Acetyllipoamide + Coenzyme A --> Acetyl CoA | Pyruvate Dehydrogenase Complex |
| Dihyrolipoyl dehydrogenase (E3) | FAD + e --> FADH2 + Lipoamide ---[NAD+]---> NADH + (H+) + FAD | Pyruvate Dehydrogenase Complex |
| Citrate Synthase | Acetyl CoA + H2O + Oxaloacetate --> Citrate (C6) | TCA Cycle |
| Aconitase | Citrate --> Isocitrate (C6) | TCA Cycle |
| Isocitrate Dehydrogenase | Isocitrate --> alpha-Ketoglutorate (C5) | TCA Cycle |
| alpha-Ketoglutorate Dehydrogenase | alpha-Ketoglutorate --> Succinyl CoA (C4) | TCA Cycle |
| Succinyl CoA Synthetase | Succinyl CoA --> Succinate (C4) | TCA Cycle |
| Succinate Dehydrogenase | Succinate --> Fumarate (C4) | TCA Cycle |
| Fumarase | Fumarate --> Malate (C4 | TCA Cycle |
| Malate Dehydrogenase | Malate --> Oxaloacetate (C4) | TCA Cycle |
| Phosphoglucomutase | G-1-P <---> G-1,6-P <---> G-6-P | Glycogen Synthesis |
| UDP-glucose Pyrophosphorylase | G-1-P + UTP ---> UDP-Glucose + PPi | Glycogen Synthesis |
| Glycogenin | Glucose + AA ---> dimer w/ Glycogen Synthase ---> +8 glucose residues ---> Glycogen Synthases finishes | Glycogen Synthesis |
| Glycogen Synthase | Glycogen(n) + UDP-glucose ---> UDP + Glycogen(n+1) | Glycogen Synthesis |
| Branching Enzyme | Cuts 7 terminal units off ---> forms alpha 1-6 linkage 4 units back | Glycogen Synthesis |
| G protein | +Adenylate cyclase | Glycogen Synthesis |
| Adenylate cyclase | ATP ---> cyclic AMP | Glycogen Synthesis |
| Cyclic AMP | +PKA | Glycogen Synthesis |
| PKA | +Phosphorylase kinase, -Glycogen synthase (b) | Glycogen Synthesis |
| Phosphorylase kinase | +Phosphorylase (a) | Glycogen Synthesis |
| Phosphorylase (a) | Glycogen(n) ---> Glucose 1-phosphate + Glycogen(n-1) | Glycogen Synthesis |
| PKA + Ca2+ | ++++ Phosphorylase kinase | Glycogen Synthesis |
| Protein phosphatase 1 (PP1) | -Phosphorylase kinase, -Phosphorylase (b), +Glycogen synthase | Glycogen Synthesis |
| Insulin-sensitive protein kinase | +PP1 | Glycogen Synthesis |
| PP1 | act* Glycogen synthase, inact* Phosphorylase kinase and Glycogen phosphorylase | Glycogen Synthesis |
| PKB | inact* GSK-3 | Glycogen Synthesis |
| GSK3 | inact* Glycogen synthase | Glycogen Synthesis |
| Panreatic (intestinal) lipases | TAGs ---> Fatty Acids | Lipoproteins |
| Lipoprotein lipase | cleaves TAGs (chylomicron) ---> FA + Glycerol | Lipoproteins |
| Apoprotein B-100 | tag on lipoprotein, tells it where it is from | Lipoproteins |
| Chylomicrons | fatty acid packaging ---> circulatory system ---> liver | Lipoproteins |
| VLDL | repackaging of FAs, cholesterol and esters ---> circulatory system | Lipoproteins |
| IDL | after liporprotein lipases extract FAs, VLDL --> IDL and is transported to liver | Lipoproteins |
| LDL | after liporprotein lipases extract FAs, VLDL --> LDL and is transported to liver | Lipoproteins |
| HDL | pick-up and return cholesterol to the liver | Lipoproteins |
| ACAT | packages VLDL | Lipoproteins |
| LCAT | HDL scavages cholesterol | Lipoproteins |
| PKA | HSL and perilipin | Lipoproteins |
| Perilipin | changes conformation, allows lipase to enter lipid droplet | Lipoproteins |
| HSL | enters lipid droplet and cleaves FAs from TAGs | Lipoproteins |
| Albumin | Transports FAs in circulatory system | Lipoproteins |
| Lipase | adds water to TAG ---> glycerol + FAs | Lipoproteins |
| Fatty acyl-CoA synthetase | FA + ATP <--> Acyl adenylate + PPi <--> Acyl adenylate + CoA <--> Acyl CoA + AMP | FA Oxidation |
| Caritine acyltransferase I (CAT I) | Acyl CoA + Carnitine ---> Acyl carnitine + CoA | FA Oxidation |
| Translocase | moves Acyl carnitine into Matrix and Carnitine out to cytoplasm | FA Oxidation |
| Carnitine acyltransferase II (CAT II) | Acyl carnitine + CoA ---> Acyl CoA + Carnitine | FA Oxidation |
| Acyl-CoA dehydrogenase | Acyl CoA + FAD ---> trans-delta2-Enoyl CoA | FA Oxidation |
| Enoyl-CoA hydratase | trans-delta2-Enoyl CoA + H2O ---> L-3-Hydroxyacyl CoA | FA Oxidation |
| beta-Hydroxyacyl-CoA dehydronase | L-3-Hydroxyacyl CoA + NAD+ ---> 3- Ketoacyl CoA + NADH | FA Oxidation |
| Acyl-CoA acetyltransferase (thiolase) | 3-Keyoacyl CoA + CoA ---> Acyl CoA + Acetyl CoA | FA Oxidation |
| delta3, delta2 -enoyl-CoA isomerase | converts cis-monosaturated FA ---> trans double bond, oxidizable by regular mechanism | FA Oxidation |
| Enoyl-CoA isomerase | acts on 1st db of polyunsaturated FAs and also trans-delta3 | FA Oxidation |
| 2,4-dienoyl-CoA reductase | works on the 2nd+ db bonds | FA Oxidation |
| Propionyl-CoA carboxylase | Propionyl-CoA + HCO3 + ATP ---(biotin)---> D-Methylmalonyl-CoA + ADP + Pi | Odd Chain FA |
| Methylmalonyl-CoA epimerase | D-Methylmalonyl-CoA ---> L-Methylmalonyl-CoA | Odd Chain FA |
| Methylmalonyl-CoA mutase | L-Methylmalonyl-CoA ---(B12)---> Succinyl-CoA | Odd Chain FA |
| Citrate Synthase | Acetyl-CoA + Oxaloacetate ---> Citrate | FA Biosynthesis |
| Citrate transporter | moves Citrate out of Mitochondria | FA Biosynthesis |
| Citrate Lyase | Citrate + CoA + ATP ---> Acetyl-CoA + ADP + Pi | FA Biosynthesis |
| Malate Dehydrogenase | Oxaloacetate + NADH + (H+) ---> Malate + NAD+ | FA Biosynthesis |
| Malate-alpha-ketoglutarate transporter | Brings Malate into the matrix | FA Biosynthesis |
| Malic Enzyme | Malate + NADP+ ---> NADPH + (H+) + CO2 + Pyruvate | FA Biosynthesis |
| Pyruvate transporter | brings Pyruvate into matrix | FA Biosynthesis |
| Pyruvate Carboxylase | Pyruvate + CO2 + ATP ---> Oxaloacetate + ADP +Pi | FA Biosynthesis |
| Malate Dehydrogenase | Malate + NAD+ ---> Oxaloacetate + NADH + (H+) | FA Biosynthesis |
| Acetyl-CoA carboxylase | Acetyl-CoA + HCO3 + ATP ---> Malonyl-CoA + ADP + Pi + (H+) | FA Biosynthesis |
| Malonyl-CoA-ACP transacylase | Malonyl-CoA + ACP ---> Malonly-ACP + CoA | FA Biosynthesis |
| Acetyl-CoA-ACP transacylase | Acetyl-CoA + ACP ---> Acetyl-ACP + CoA | FA Biosynthesis |
| beta-Ketoacyl synthase (condensing) | Acetyl-ACP + Malonyl-ACP ---> Acetoacetyl-ACP + ACP + CO2 | FA Biosynthesis |
| beta-Ketoacyl-ACP reductase | Acetoacetyl-ACP + NADPH ---> D-3-Hydroxbutyryl-ACP + NADP+ | FA Biosynthesis |
| beta-Hydroxyacyl-ACP dehydratase | D-3-Hydroxybutyryl-ACP ---> H2O + Crotonyl-ACP | FA Biosynthesis |
| Fatty acyl-CoA desaturase | extracts electrons from 2 diff substrates and creates a double bond (saturated fatty acyl-CoA) | FA Biosynthesis |
| Carbamoyl phosphate synthetase I (CPS I) | Bicarbonate + ATP ---> Carboxyphosphate + NH3 ---> Carbamic acid + ATP ---> Carbamoyl phosphate | Pyrimidine Synthesis |
| Aspartate trans-carbamoylase (ATCase) | Carbamoyl phosphate + Aspartate ---> Carbamoylaspartate ---> Dihydroorotate + NAD+ ---> Orotate | Pyrimidine Synthesis |
| Orotate phosphoribosyl transferase | Orotate + 5--phosphoribosyl-1-pyrophosphate (PRPP) ---> Orotidylate | Pyrimidine Synthesis |
| Orotidylate decarboxylase | Orotidylate ---> Uridylate (UMP) | Pyrimidine Synthesis |
| UMP kinase | UMP + ATP ---> UDP + ADP | Pyrimidine Synthesis |
| Nucleoside diphosphate kinase | UDP + YTP ---> UTP + YDP | Pyrimidine Synthesis |
| Glutamine (NH3) | UTP + NH3 + ATP ---> CTP + ADP + Pi | Pyrimidine Synthesis |
| PRPP synthetase | Ribose 5-phosphate ---> PRPP | Purine Synthesis |
| Glutamine phosphoribosyl amidotransferase | PRPP + Glutamine ---> 5-Phospho-beta-D-ribosylamine | Purine Synthesis |
| IMP dehydrogenase | IMP ---> Xanthylate (XMP) + NH3 + ATP ---> Guanylate (GMP) | Purine Synthesis |
| Adenylosuccinate synthetase | IMP + GTP + Aspartate ---> Adenylsuccinate ---> Adenylate (AMP) | Purine Synthesis |
| HMG-CoA synthase | Acetoacetyl-CoA + Acetyl-CoA ---> HMG-CoA + CoA | Cholesterol Synthesis |
| HMG-CoA reductase | HMG-CoA ---> Mevalonate | Cholesterol Synthesis |
| Squalene synthase | Farnesyl pyrophosphate + Farnesyl pyrophosphate ---> Squalene (C30) | Cholesterol Synthesis |
| 19 steps | Lanosterol --(19 steps)--> Cholesterol | Cholesterol Synthesis |
| ACAT | Cholesterol ---> Cholesteryl ester (LDL form) | Cholesterol Synthesis |
| Glutaminase | Glumanine ---> Glutamate + NH4+ | AA Synthesis |
| Amino transferase + (PlP) | alpha-Ketoglutarate + Amino Acid ---> Glutamate + alpha-Keto acid | AA Synthesis |
| ALT | Pyruvate + Glutamate <---> Alanine (blood) + alpha-Ketoglutarate [moves amine between mucle and liver] | Cahill Cycle |
| Glutamate dehydrogenase | Glutamate <-[schiff base]--> NH4+ + alpha-Ketoglutarate | Urea Cycle |
| Carbamoyl phosphate synthetase-1 | Bicarb + 2ATPs ---> ---> Carbamoyl phosphate | Urea Cycle-Mito |
| Ornithine transcarbamoylase | Orthine +Carbamoyl phosphate ---> Citrulline | Urea Cycle-Mito |
| Argininosuccinate synthetase | Citrulline + Aspartate + 2ATPS ---> Argininosuccinate | Urea Cycle-Cyto |
| Argininosuccinase | Argininosuccinate ---> Arginine + Fumarate | Urea Cycle-Cyto |
| Arginase | Arginine ---> Ornithine + Urea | Urea Cycle-Cyto |
| Pyridoxal phosphate (PLP) | co-enzyme for aminotransferase = transamination | Urea Cycle |
| Pyridoxamine phosphate | co-enzyme for aminotransferase = transamination | Urea Cycle |
| Glutamate dehydrogenase | Glutamate ---> [Schiff base] ---> alpha-Ketoglutarate + NH4+ | Urea Cycle |
| ALT (PLP) | Tryptophan ---> Alanine ---> Pyruvate | AA Catabolism |
| Serine dehydratase (PLP) | Serine ---> Pyruvate | AA Catabolism |
| AA | Cysteine ---> Pyruvate | AA Catabolism |
| Serine hydroxylmethyl transferase | Threonine ---> Serine + Folate | AA Catabolism |
| Glycine cleavage enzyme | Glycine ---> NH4+ Folate | AA Catabolism |
| Phenylalanine hydroxylase (PKU) | Phenylalanine ---> Tyrosine | AA Catabolism |
| Homogentisate 1,2-dioxygenase (Alkaptonuria) | Homogentisate ---> Maleylacetoacetate | AA Catabolism |
| Glutamate dehydrogenase | a-Ketoglutarate ---> Glutamate | AA Synthesis |
| Glutamine synthetase | Glutatmate ---> Glutamine | AA Synthesis |
| ALT | Pyruvate ---> Alanine | AA Synthesis |
| AST | Oxaloacetate ---> Aspartate | AA Synthesis |
| Glutamine(NH3) | Aspartate ---> Asparagine | AA Synthesis |
| (no enzyme) | Glutamate ---> Ornithine <---> Proline <---> Ornithine <--- Arginine | AA Synthesis |
| (no enzyme) | 3-Phosphoglycerate ---> 3-Phosphohydroxyl pyruvate ---> 3-phosphoserine ---> Serine | AA Synthesis |
| Serine hydroxylmethylase (PLP) | Serine + THF ---> Glycine + 5,10-methylTHF | AA Synthesis |
| Glycine synthase | CO2 + NH4 + 5,10-MethylTHF ---> Glycine + THF | AA Synthesis |
| SAM | Met + ATP ---> SAM | AA Synthesis |
| SAM | SAM + S-Ahomocysteine ---> Homocysteine | AA Synthesis |
| Methionine synthase (B12) | Homocysteine + N5-MethylTHF ---> Methionine + THF | AA Synthesis |
| none | Homocysteine + Serine ---> ---> Cysteine + a-Ketobutyrate | AA Synthesis |
| Tyrosine hydroxylase | Tyrosine ---> L-Dopa | Catacholamine Synthesis |
| Aromatic AA decarboxylase | L-Dopa ---> Dopamine | Catacholamine Synthesis |
| Dopamine hydroxylase | Dopamine ---> NorEpi | Catacholamine Synthesis |
| Ph-N-Methyltransferase | NorEpi ---> Epi | Catacholamine Synthesis |
| Aromatic AA decarboxylase | Tryptophan ---> Serotonin | Catacholamine Synthesis |
| Glutamate decarboxylase | Glutamate ---> GABA | Catacholamine Synthesis |
| Histadine decarboxylase | Histadine ---> Histamine | Catecholamine Synthesis |
| heme syn 1 | Succinyl-CoA + Glycine ---> g-Aminolevulinate | Heme Synthesis |
| heme syn 2 | (2) g-Aminolevulinate ---> Porphobilogen | Heme Synthesis |
| Porphobilinogen deaminase | Porphobilogen ---> linear Terapyrrole + 4NH3+ | Heme Synthesis |
| Uroporphyrinogen synthase + (uroporphyrinogen III) | Uroporphyrinogen III (isoenzyme) | Heme Synthesis |
| heme syn 5 | Uroporphyrinogen III + Methyl ---> Coproporphyrinogen III + Vinyl ---> Protoporhyrin IX | Heme Synthesis |
| Ferrochetalase | Protoporhyrin IX + Fe ---> Heme | Heme Synthesis |
| Heme oxygenase | Heme ---> Biliverdin + CO- | Heme Degradation |
| Biliverdin reductase | Biliverdin ---> Bilirubin | Heme Degradation |
| Glucuronyl-bilirubin transferase (liver) | Bilirubin changed - transported to intestine - changed back to Bilirubin (bile) ---> Urobilinogen ==> Stercobilin (feces), Urobilin (kidney-urine) | Heme Degradation |
| g-Glutanyl cysteine synthetase | Glutamate + Cystein ---> g-Glu-Cys | Glutathione Synthesis |
| Glutathione synthetase | g-Glu-Cys + Glycine ---> Glutathione (GSH) | Glutathione Synthesis |
| Glutathione peroxidase | GSH ---> GSSG (glutathione dimer w/ disulfide bridge) | Glutathione Synthesis |
| Glutathione reductase | GSSG ---> GSH | Glutathione Synthesis |
| Creatine kinase | Creatine phosphate + ADP <----> ATP + Creatine | Glutathione Synthesis |
| Ribonucleotide reductase | Ribonucleoside diphosphate ---> Deoxyribonucleoside diphosphate | Deoxyribonucleotide Syn |
| Thymidylate synthase | dUMP + 5,10-MethylTHF ---> dTMP + Dihydrofolate | Deoxyribonucleotide Syn |
| Dihydrofolate reductase | Dihydrofolate + NADPH ---> THF + NADP+ | Deoxyribonucleotide Syn |
| none | THF + Serine ---> 5,10-MethylTHF + Glycine | Deoxyribonucleotide Syn |
| Fluorodeoxyuridylate | Thymidylate synthase inhibitor | Deoxyribonucleotide Syn |
| Aminopterin & methotrexate | Dihydrofolate reductase inhibitor | Deoxyribonucleotide Syn |
| Adenine phosphoribosyltransferase | Adenine + PRPP ---> Adenylate + PPi | Purine Salvage Pathway |
| Hypoxanthine-guanine phosphoribosyltransferase (HGPRT) | Hypoxanthine + PRPP ---> inosinate + PPi | Puine Salvage Pathway |
| HGPRT | Guanine + PRPP ---> Guanylate + PPi | Purine Salvage Pathway |
| Orotate phosphoribosyl transferase | Orotate + PRPP ---> Orotidylate | Pyrimidine Salvage Pathway |
| Xanthine Oxidase | AMP ---> IMP ---> Hypoxanthine ---> Xanthine ---> Uric acid ---> Urate | Purine Breakdown |
| Guanase | Guanine ---> Xanthine ---> Uric acid ---> Urate | Purine Breakdown |
| Allopurinol | Xanthine oxidase inhibitor = gout treatment | Gout treatment |
| Xanthine oxidase | Allopurinol ---> Alloxanthine | Gout treatment |