methionine adenosyltransferase 1A
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The MAT1A gene provides instructions for producing the enzyme methionine adenosyltransferase. The enzyme is produced from the MAT1A gene in two forms, designated MATI and MATII. MATI is made up of four identical protein subunits, which is known as a homotetramer. MATII is made up of two of the same protein subunits, which is known as a homodimer. Both forms of the enzyme are found in the liver.
Normal function
The MAT1A gene provides instructions for producing the enzyme methionine adenosyltransferase. The enzyme is produced from the MAT1A gene in two forms, designated MATI and MATII. MATI is made up of four identical protein subunits, which is known as a homotetramer. MATII is made up of two of the same protein subunits, which is known as a homodimer. Both forms of the enzyme are found in the liver.
Both the MATI and MATII forms of methionine adenosyltransferase help break down a protein building block (amino acid) called methionine. The enzyme starts the reaction that converts methionine to S-adenosylmethionine, also called AdoMet or SAMe. AdoMet is involved in transferring methyl groups, consisting of a carbon atom and three hydrogen atoms, to other compounds, a process called transmethylation. Transmethylation is important in many cellular processes. These processes include determining whether the instructions in a particular segment of DNA are carried out, regulating reactions involving proteins and lipids, and controlling the processing of chemicals that relay signals in the nervous system (neurotransmitters).
Conditions referring to this gene or chromosome
Other names
MAT
MATA1
methionine adenosyltransferase I, alpha
METK1_HUMAN
S-adenosylmethionine synthetase 1
SAMS
SAMS, liver-specific
SAMS1
Leitura adicional e referências
- methionine adenosyltransferase 1A; MedlinePlus, National Library of Medicine.
- Biochemistry (fifth edition, 2002): Methionine Metabolism
- Chamberlin ME, Ubagai T, Mudd SH, Thomas J, Pao VY, Nguyen TK, Levy HL, Greene C, Freehauf C, Chou JY. Methionine adenosyltransferase I/III deficiency: novel mutations and clinical variations. Am J Hum Genet. 2000 Feb;66(2):347-55. doi: 10.1086/302752.
- Chien YH, Chiang SC, Huang A, Hwu WL. Spectrum of hypermethioninemia in neonatal screening. Early Hum Dev. 2005 Jun;81(6):529-33. doi: 10.1016/j.earlhumdev.2004.11.005. Epub 2004 Dec 19.
- Chou JY. Molecular genetics of hepatic methionine adenosyltransferase deficiency. Pharmacol Ther. 2000 Jan;85(1):1-9. doi: 10.1016/s0163-7258(99)00047-9.
- Smythies JR, Gottfries CG, Regland B. Disturbances of one-carbon metabolism in neuropsychiatric disorders: a review. Biol Psychiatry. 1997 Jan 15;41(2):230-3. doi: 10.1016/S0006-3223(96)00068-6. No abstract available.
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