| URN | urn:agi-llid:4552 |
|---|---|
| Connectivity | 26 |
| Name | Mtrr |
| Description | 5-methyltetrahydrofolate-homocysteine methyltransferase reductase |
| Notes | Methionine is an essential amino acid required for protein synthesis and one-carbon metabolism. Its synthesis is catalyzed by the enzyme methionine synthase. Methionine synthase eventually becomes inactive due to the oxidation of its cob(I)alamin cofactor. The protein encoded by this gene regenerates a functional methionine synthase via reductive methylation. It is a member of the ferredoxin-NADP(+) reductase (FNR) family of electron transferases. Patients of the cbl-E complementation group of disorders of folate/cobalamin metabolism are defective in reductive activation of methionine synthase. Alternative splicing of this gene results in multiple transcript variants encoding distinct isoforms. [provided by RefSeq] |
| Pathway | methionine cycle/metabolic pathway |
|---|---|
| homocysteine metabolic pathway | |
| folate cycle metabolic pathway | |
| folated mediated one carbon metabolic pathway | |
| methionine cycle-metabolic pathway |
| GO Molecular Function | oxidoreductase activity |
|---|---|
| transferase activity | |
| FMN binding | |
| NADP or NADPH binding | |
| FAD binding | |
| iron ion binding | |
| methyltransferase activity | |
| aquacobalamin reductase activity | |
| [methionine synthase] reductase activity |
| GO Cellular Component | cytoplasm |
|---|---|
| cytosol | |
| mitochondrion |
| GO Biological Process | oxidation reduction |
|---|---|
| methionine metabolic process | |
| methionine biosynthetic process | |
| cellular amino acid biosynthetic process | |
| protein stabilization |
| Ariadne Ontology | Folate and pterins biosynthesis |
|---|
| Group | Folate and pterins biosynthesis |
|---|---|
| oxidation reduction | |
| methionine metabolic process | |
| methionine biosynthetic process | |
| cellular amino acid biosynthetic process | |
| protein stabilization | |
| oxidoreductase activity | |
| transferase activity | |
| FMN binding | |
| NADP or NADPH binding | |
| FAD binding | |
| iron ion binding | |
| methyltransferase activity | |
| aquacobalamin reductase activity | |
| [methionine synthase] reductase activity | |
| cytoplasm | |
| cytosol | |
| mitochondrion |
| MedScan ID | 4552 |
|---|
| Hugo ID | 7473 |
|---|
| Human chromosome position | 5p15.3-p15.2 |
|---|
| LocusLink ID | 4552 |
|---|---|
| 210009 | |
| 290947 |
| Alias | MSR |
|---|---|
| cblE | |
| MGC129643 | |
| methionine synthase reductase | |
| methionine synthase reductase, mitochondrial | |
| [methionine synthase]-cobalamin methyltransferase (cob(II)alamin reducing) | |
| 4732420G08 | |
| MGC112912 | |
| (methionine synthase)-cobalamin methyltransferase (cob(II)alamin reducing) | |
| (methionine synthetase)-cobalamin methyltransferase (cob(II)alamin reducing) | |
| 5-methyltetrahydrofolate-homocysteine methyltransferase reductase | |
| methionine synthase reductase isoform 1 | |
| methionine synthase reductase isoform 2 | |
| methionine synthase reductase isoform I | |
| methionine synthase reductase isoform II | |
| methionine synthetase reductase | |
| methionine synthetase reductase isoform 1 | |
| methionine synthetase reductase isoform 2 | |
| methionine synthetase reductase isoform I | |
| methionine synthetase reductase isoform II | |
| Methionine synthetase reductase, mitochondrial | |
| mitochondrial similar to Methionine synthase reductase precursor (MSR) | |
| mitochondrial similar to Methionine synthetase reductase precursor (MSR) | |
| MTRR |
| Organism | Homo sapiens |
|---|---|
| Mus musculus | |
| Rattus norvegicus |
| OMIM ID | 602568 |
|---|---|
| 236270 | |
| 601634 |
| Mouse chromosome position | 13 41.0 cM |
|---|
| GO ID | 0050660 |
|---|---|
| 0010181 | |
| 0050661 | |
| 0030586 | |
| 0005506 | |
| 0016491 | |
| 0008652 | |
| 0009086 | |
| 0006555 | |
| 0055114 | |
| 0005737 | |
| 0005829 | |
| 0047138 | |
| 0008168 | |
| 0016740 | |
| 0050821 | |
| 0005739 |
| Rat chromosome position | 17p14 |
|---|
| Swiss-Prot Accession | Q7Z4M8 |
|---|---|
| Q9UBK8 | |
| Q8C1A3 | |
| Q3U2C6 | |
| Q8R0Y3 | |
| Q498R1 | |
| O60471 | |
| Q32MA9 |
| Unigene ID | Hs.481551 |
|---|---|
| Mm.480007 | |
| Rn.59092 |
| KEGG ID | hsa:4552 |
|---|---|
| mmu:210009 | |
| rno:290947 |
| EC Number | 1.16.1.8 |
|---|
| Swiss-Prot ID | MTRR_HUMAN |
|---|---|
| Q8C1A3_MOUSE | |
| Q498R1_RAT | |
| Q7Z4M8_HUMAN |
| Cell Localization | Cytoplasm |
|---|---|
| Mitochondria |
| IPI ID | IPI00221302 |
|---|---|
| IPI00221303 | |
| IPI00307334 | |
| IPI00272780 | |
| IPI00365431 | |
| IPI00889777 | |
| IPI00780056 |
| Homologene ID | 11419 |
|---|
| RGD ID | 1308671 |
|---|
| MGI ID | 1891037 |
|---|