Protein Mtrr

URN	urn:agi-llid:4552
Total Entities	0
Connectivity	177
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]

GO Molecular Function	oxidoreductase activity
	oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, NAD(P)H as one donor, and incorporation of one atom of oxygen
	NADPH-hemoprotein reductase activity
	oxidoreductase activity, oxidizing metal ions, NAD or NADP as acceptor
	[methionine synthase] reductase activity
	aquacobalamin reductase activity
	flavin adenine dinucleotide binding
	NADP binding
	FMN binding
	iron ion binding

GO Cellular Component	nucleolus
	cytoplasm
	cytosol
	intermediate filament cytoskeleton
	nucleus

GO Biological Process	small molecule metabolic process
	oxidation-reduction process
	cellular amino acid biosynthetic process
	methionine biosynthetic process
	vitamin metabolic process
	sulfur amino acid metabolic process
	folic acid metabolic process
	methionine metabolic process
	water-soluble vitamin metabolic process
	cobalamin metabolic process
	xenobiotic metabolic process
	DNA methylation
	protein stabilization
	methylation
	cellular nitrogen compound metabolic process

Pathway	homocysteine metabolic pathway

Group	Folate and pterins biosynthesis
	small molecule metabolic process
	oxidation-reduction process
	cellular amino acid biosynthetic process
	methionine biosynthetic process
	oxidoreductase activity
	oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, NAD(P)H as one donor, and incorporation of one atom of oxygen
	NADPH-hemoprotein reductase activity
	oxidoreductase activity, oxidizing metal ions, NAD or NADP as acceptor
	[methionine synthase] reductase activity
	aquacobalamin reductase activity
	vitamin metabolic process
	sulfur amino acid metabolic process
	folic acid metabolic process
	methionine metabolic process
	water-soluble vitamin metabolic process
	cobalamin metabolic process
	xenobiotic metabolic process
	DNA methylation
	protein stabilization
	methylation
	cellular nitrogen compound metabolic process
	flavin adenine dinucleotide binding
	NADP binding
	FMN binding
	iron ion binding
	nucleolus
	cytoplasm
	cytosol
	intermediate filament cytoskeleton
	nucleus
	Secreted proteins

Cell Localization	Mitochondria
	Cytoplasm

Alias	MSR
	cblE
	methionine synthase reductase
	methionine synthase reductase, mitochondrial
	[methionine synthase]-cobalamin methyltransferase (cob(II)alamin reducing)
	4732420G08
	mitochondrial similar to Methionine synthetase reductase precursor (MSR)
	methionine synthetase reductase isoform 2
	methionine synthetase reductase isoform I
	methionine synthetase reductase isoform II
	MTRR
	Methionine synthetase reductase, mitochondrial
	MGC112912
	mitochondrial similar to Methionine synthase reductase precursor (MSR)
	methionine synthetase reductase isoform 1
	MGC129643
	(methionine synthetase)-cobalamin methyltransferase (cob(II)alamin reducing)
	methionine synthetase reductase
	(methionine synthase)-cobalamin methyltransferase (cob(II)alamin reducing)
	5-methyltetrahydrofolate-homocysteine methyltransferase reductase
	methionine synthase reductase isoform 1
	methionine synthase reductase isoform I
	methionine synthase reductase isoform II
	methionine synthase reductase isoform 2

Mouse chromosome position	13 35.54 cM
	13 41.0 cM

Rat chromosome position	1
	17p14

Hugo ID	7473

Human chromosome position	5p15.31
	5p15.3-p15.2

EC Number	1.16.1.8

Homologene ID	11419

MGI ID	1891037

RGD ID	1308671

MedScan ID	4552