SMPDB_ID

SMP0000221

PW_ID

PW000105

图片

主题

Disease

描述

Methionine adenosyltransferase (MAT; Hypermethioninemia; MAT I/III deficiency) deficiency is caused by mutations in the MAT1A gene which causes isolated hypermethioninemia. MAT catalyzes the formation of adenosylmethionine from methionine and ATP. Adenosylmethionine is an important methyl donor in most transmethylation reactions. MAT dificiency is characterized by increased homocysteine and methionine levels in plasma; and accumulation of methionine in urine. Symptoms include dystonia, mental retardation and unusual odor.

代谢物

SMPDB ID	代谢物id	代谢物的名字
SMP0000221	PW_C009795	Zinc
SMP0000221	PW_C000457	Potassium
SMP0000221	PW_C000423	Magnesium
SMP0000221	PW_C001342	Methionine sulfoxide
SMP0000221	PW_C000774	10-Formyltetrahydrofolate
SMP0000221	PW_C000170	Pyrophosphate
SMP0000221	PW_C000032	Adenosine monophosphate
SMP0000221	PW_C000414	Adenosine triphosphate
SMP0000221	PW_C001783	Hydrogen peroxide
SMP0000221	PW_C000035	Ammonia
SMP0000221	PW_C001195	2-Oxo-4-methylthiobutanoic acid
SMP0000221	PW_C001065	Oxygen
SMP0000221	PW_C000062	Dimethylglycine
SMP0000221	PW_C000030	Betaine
SMP0000221	PW_C000065	Choline
SMP0000221	PW_C000034	Adenosine
SMP0000221	PW_C000770	5-Methylthioribose 1-phosphate
SMP0000221	PW_C000971	Spermidine
SMP0000221	PW_C000910	5'-Methylthioadenosine
SMP0000221	PW_C001092	Putrescine
SMP0000221	PW_C001316	Carbon dioxide
SMP0000221	PW_C000783	S-Adenosylmethioninamine
SMP0000221	PW_C000185	Sarcosine
SMP0000221	PW_C000749	S-Adenosylhomocysteine
SMP0000221	PW_C000921	S-Adenosylmethionine
SMP0000221	PW_C000003	2-Ketobutyric acid
SMP0000221	PW_C000448	L-Cysteine
SMP0000221	PW_C000067	L-Cystathionine
SMP0000221	PW_C000570	L-Homoserine
SMP0000221	PW_C001005	Zinc (II) ion
SMP0000221	PW_C000548	L-Methionine
SMP0000221	PW_C000590	Homocysteine
SMP0000221	PW_C000964	FAD
SMP0000221	PW_C000721	NAD
SMP0000221	PW_C001079	5-Methyltetrahydrofolic acid
SMP0000221	PW_C001144	NADH
SMP0000221	PW_C001148	Pyridoxal 5'-phosphate
SMP0000221	PW_C001420	Water
SMP0000221	PW_C000078	Glycine
SMP0000221	PW_C001178	5,10-Methylene-THF
SMP0000221	PW_C000120	L-Serine
SMP0000221	PW_C001221	Tetrahydrofolic acid
SMP0000221	PW_C001104	Phosphate

蛋白质

SMPDB ID	蛋白质id	蛋白质的名字
SMP0000221	Q9Y3D2	Methionine-R-sulfoxide reductase B2, mitochondrial
SMP0000221	Q8IXL7	Methionine-R-sulfoxide reductase B3
SMP0000221	Q8NE62	Choline dehydrogenase, mitochondrial
SMP0000221	Q9NZL9	Methionine adenosyltransferase 2 subunit beta
SMP0000221	P31153	S-adenosylmethionine synthase isoform type-2
SMP0000221	Q96DP5	Methionyl-tRNA formyltransferase, mitochondrial
SMP0000221	P56192	Methionine--tRNA ligase, cytoplasmic
SMP0000221	Q96RQ9	L-amino-acid oxidase
SMP0000221	P23526	Adenosylhomocysteinase
SMP0000221	Q13126	S-methyl-5'-thioadenosine phosphorylase
SMP0000221	P19623	Spermidine synthase
SMP0000221	P17707	S-adenosylmethionine decarboxylase proenzyme
SMP0000221	P26358	DNA (cytosine-5)-methyltransferase 1
SMP0000221	Q93088	Betaine--homocysteine S-methyltransferase 1
SMP0000221	P35520	Cystathionine beta-synthase
SMP0000221	P32929	Cystathionine gamma-lyase
SMP0000221	Q99707	Methionine synthase
SMP0000221	P42898	Methylenetetrahydrofolate reductase
SMP0000221	P34896	Serine hydroxymethyltransferase, cytosolic