| Enzyme Name | Gene Symbol | Functions / Comments |
| Aconitases | ACO1, ACO2 | the protein encoded by ACO1 functions in the iron-mediated control of translation of the H-ferritin, L-ferritin, transferrin receptor, DMT1, ferroportin, ALAS2, and ACO2 mRNAs; the ACO2 encoded protein is involved in the TCA cycle |
| Alcohol dehydrogenases | 7 different genes | belong to medium-chain dehydrogenase/reductase (MDR) superfamily; catalyze the oxidation of various alcohols to their corresponding aldehydes; important in the detoxification/metabolism of ethanol |
| Catalase | CAT | primary reaction is to detoxify the reactive oxygen species (ROS) hydrogen peroxide (H2O2) to water; can also oxidize certain alcohols to corresponding aldehydes |
| Cytochrome c reductase | multiple subunits including the Fe-S protein encoded by the UQCRFS1 gene | multisubunit component of oxidative phosphorylation Complex III; contains two cytochromes b (b-562 and b-566), cytochrome c1, and the Fe-S protein which is called the Rieske Fe-S protein after its discoverer J.S. Rieske; official name of this enzyme complex is ubiquinol-cytochrome c reductase |
| Lipoxygenases | ALOX5, ALOX12, ALOX15 | all three lipoxygenases (5-LOX, 12-LOX, and 15-LOX) are involved in arachidonic acid oxidation during the synthesis of the leukotrienes and the lipoxins |
| Lysyl hydroxylases | PLOD1, PLOD2, PLOD3 | official name for these enzymes is procollagen-lysine, 2-oxoglutarate 5-dioxygenase; PLOD1 is the major procollagen lysine hydroxylating enzyme; all 3 enzymes function as homodimers; PLOD2 and PLOD3 carry out hydroxylations in collagen-like proteins; mutations in PLOD1 are associated with Ehlers-Danlos syndrome (EDS) type VI, mutations in PLOD2 or PLOD3 are associated with EDS type VIB |
| NADH-ubiquinone reductase | multiple Fe-S subunit genes | multisubunit component of oxidative phosphorylation Complex I |
| Phenylalanine hydroxylase | PAH | catalyzes the conversion of phenylalanine to tyrosine; mutations in the PAH gene result in phenylketonuria, PKU |
| Prolyl 4-hydroxylase (two α, two β subunits) | three α subunit genes: P4HA1, P4HA2, P4HA3; β subunit gene: P4HB | catalyzes the formation of 4-hydroxyproline residues in procollagen |
| Ribonucleotide reductase (contains 2 subunits) | RRM1, RRM2 | catalyzes the conversion of ribonucleoside diphosphates to their corresponding deoxyribonucleotide diphosphates |
| Stearoyl-CoA desaturase | SCD | one of three fatty acid desaturases in humans; stearoyl-CoA desaturase is the rate-limiting enzyme catalyzing the synthesis of monounsaturated fatty acids (MUFAs), primarily oleate (18:1; a physiologically significant omega-9 fatty acid) and palmitoleate (16:1) |
| Succinate-ubiquinone reductase | multiple Fe-S subunit genes | multisubunit component of oxidative phosphorylation Complex II |
| Thyroid peroxidase | TPO | exclusively expressed in the thyroid gland; within the thyroid colloid TPO oxidizes I– to I+; reaction requires H2O2 |
| Tryptophan hydroxylase | TPH2 | initial enzyme in the conversion of tryptophan to the neurotransmitters, serotonin and melatonin |
| Tyrosine hydroxylase | TH | initial enzyme in the conversion of tyrosine to the catecholamines, dopamine, norepinephrine and epinephrine |
| Xanthine oxidase (derived from xanthine dehydrogenase) | XDH | also requires molybdenum for function; xanthine dehydrogenase can be converted to xanthine oxidase by reversible sulfhydryl oxidation or by irreversible proteolytic modification; catalyzes the conversion of hypoxanthine to xanthine and xanthine to uric acid in the catabolism and salvage of purine nucleotides |
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