Names & Taxonomy

Uniprot ID:
P42898
Entry Name:
MTHR_HUMAN
Status:
reviewed
Protein Names:
Methylenetetrahydrofolate reductase (EC 1.5.1.20)
Gene Names:
MTHFR
Gene Names Primary:
MTHFR
Organism:
Homo sapiens (Human)

Structure

Length:
656
Sequence:
MVNEARGNSSLNPCLEGSASSGSESSKDSSRCSTPGLDPERHERLREKMRRRLESGDKWFSLEFFPPRTAEGAVNLISRFDRMAAGGPLYIDVTWHPAGDPGSDKETSSMMIASTAVNYCGLETILHMTCCRQRLEEITGHLHKAKQLGLKNIMALRGDPIGDQWEEEEGGFNYAVDLVKHIRSEFGDYFDICVAGYPKGHPEAGSFEADLKHLKEKVSAGADFIITQLFFEADTFFRFVKACTDMGITCPIVPGIFPIQGYHSLRQLVKLSKLEVPQEIKDVIEPIKDNDAAIRNYGIELAVSLCQELLASGLVPGLHFYTLNREMATTEVLKRLGMWTEDPRRPLPWALSAHPKRREEDVRPIFWASRPKSYIYRTQEWDEFPNGRWGNSSSPAFGELKDYYLFYLKSKSPKEELLKMWGEELTSEESVFEVFVLYLSGEPNRNGHKVTCLPWNDEPLAAETSLLKEELLRVNRQGILTINSQPNINGKPSSDPIVGWGPSGGYVFQKAYLEFFTSRETAEALLQVLKKYELRVNYHLVNVKGENITNAPELQPNAVTWGIFPGREIIQPTVVDPVSFMFWKDEAFALWIERWGKLYEEESPSRTIIQYIHDNYFLVNLVDNDFPLDNCLWQVVEDTLELLNRPTQNARETEAP
Proteomes:
UP000005640

Function

Function:
Catalyzes the conversion of 5,10-methylenetetrahydrofolate to 5-methyltetrahydrofolate, a co-substrate for homocysteine remethylation to methionine.
Pathway:
One-carbon metabolism; tetrahydrofolate interconversion.
Catalytic Activity:
5-methyltetrahydrofolate + NAD(P)(+) = 5,10-methylenetetrahydrofolate + NAD(P)H.
Cofactor:
COFACTOR: Name=FAD; Xref=ChEBI:CHEBI:57692;
Enzyme Regulation:
ENZYME REGULATION: Allosterically regulated by S-adenosylmethionine.
Active Site:
ACT_SITE 63 63 Proton donor/acceptor.
Cross Reference Drug Bank:
DB00542 DB00115 DB00544 DB00158 DB00134 DB00170 DB00563 DB00140 DB00116
Gene Ontology Go:
cytosol
neuron projection
flavin adenine dinucleotide binding
methylenetetrahydrofolate reductase (NAD(P)H) activity
modified amino acid binding
NADP binding
protein complex binding
blood circulation
cellular amino acid metabolic process
folic acid metabolic process
heterochromatin maintenance
homocysteine metabolic process
methionine metabolic process
regulation of histone methylation
response to drug
response to folic acid
response to hypoxia
response to interleukin-1
response to vitamin B2
S-adenosylmethionine metabolic process
small molecule metabolic process
tetrahydrofolate interconversion
vitamin metabolic process
water-soluble vitamin metabolic process
Gene Ontology Biological Process:
blood circulation
cellular amino acid metabolic process
folic acid metabolic process
heterochromatin maintenance
homocysteine metabolic process
methionine metabolic process
regulation of histone methylation
response to drug
response to folic acid
response to hypoxia
response to interleukin-1
response to vitamin B2
S-adenosylmethionine metabolic process
small molecule metabolic process
tetrahydrofolate interconversion
vitamin metabolic process
water-soluble vitamin metabolic process
Gene Ontology Molecular Function:
flavin adenine dinucleotide binding
methylenetetrahydrofolate reductase (NAD(P)H) activity
modified amino acid binding
NADP binding
protein complex binding
Gene Ontology Cellular Component:
cytosol
neuron projection
Keywords:
Allosteric enzyme
Alternative splicing
Complete proteome
Disease mutation
FAD
Flavoprotein
NADP
Oxidoreductase
Polymorphism
Reference proteome

Publication

PubMed ID:
9680386 14702039 16710414 15489334 7920641 12370778 21269460 7726158 7564788 7647779 8826441 8940272 8895734 9545406 9545395 9781030 9719624 10323741 10536004 10679944 15534175 18987736 20236116 25736335