Betaine--homocysteine S-methyltransferase 1

Function

Involved in the regulation of homocysteine metabolism. Converts betaine and homocysteine to dimethylglycine and methionine, respectively. This reaction is also required for the irreversible oxidation of choline.

Classification

Family (Pfam)

PF02574 S-methyl_trans

InterPro

BHMT, BHMT-like, HCY_dom, HCY_dom_sf

Functional cluster

Mixed Regulatory & Membrane Proteins

Experimental structures · PDB · 4

• 1LT7 X-ray 2.15A
• 1LT8 X-ray 2.05A
• 4M3P X-ray 1.90A
• 8D45 EM 2.62A

A predicted model is available from AlphaFold.

Gene Ontology · 10

• GO:0005829 cytosol
• GO:0070062 extracellular exosome
• GO:0005634 nucleus
• GO:0047150 betaine-homocysteine S-methyltransferase activity
• GO:0008270 zinc ion binding
• GO:0071266 'de novo' L-methionine biosynthetic process
• GO:0006579 amino-acid betaine catabolic process
• GO:0006577 amino-acid betaine metabolic process
• GO:0071267 L-methionine salvage
• GO:0032259 methylation

Drugs targeting this protein · 1

• BETAINE substrate

Related proteins · sequence + function similarity

• Betaine--homocysteine S-methyltransferase 1 1.00
• Betaine--homocysteine S-methyltransferase 1 0.99
• Betaine--homocysteine S-methyltransferase 1 0.99
• Betaine--homocysteine S-methyltransferase 1 0.98
• Betaine--homocysteine S-methyltransferase 1 0.97
• Betaine--homocysteine S-methyltransferase 1 0.96
• Betaine--homocysteine S-methyltransferase 1 0.93
• Betaine--homocysteine S-methyltransferase 1 0.91
• S-methylmethionine--homocysteine S-methyltransferase BHMT2 0.86
• S-methylmethionine--homocysteine S-methyltransferase BHMT2 0.86
• S-methylmethionine--homocysteine S-methyltransferase BHMT2 0.77
• S-methylmethionine--homocysteine S-methyltransferase BHMT2 0.76

Co-cited proteins · studied together in the literature

• Betaine--homocysteine S-methyltransferase 1 2 shared papers

Literature · 9 cited papers

• An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome. J. Proteomics · 2014
• The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. · 2004
• Investigations of a common genetic variant in betaine-homocysteine methyltransferase (BHMT) in coronary artery disease. Atherosclerosis · 2003
• Betaine-homocysteine methyltransferase: zinc in a distorted barrel. Structure · 2002
• Recombinant human liver betaine-homocysteine S-methyltransferase: identification of three cysteine residues critical for zinc binding. Biochemistry · 1999
• Interaction between dietary methionine and methyl donor intake on rat liver betaine-homocysteine methyltransferase gene expression and organization of the human gene. J. Biol. Chem. · 1999
• Human betaine-homocysteine methyltransferase is a zinc metalloenzyme. Arch. Biochem. Biophys. · 1998
• Betaine-homocysteine methyltransferase expression in porcine and human tissues and chromosomal localization of the human gene. Arch. Biochem. Biophys. · 1997
• Purification, kinetic properties, and cDNA cloning of mammalian betaine-homocysteine methyltransferase. J. Biol. Chem. · 1996