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Home / Antibodies

DMGDH Antibody

SKU : BHA10558379

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CUSABIO TECHONOLOGY LLC

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Mfr.No.	CSB-PA699345
Alternative Names	dimethylglycine dehydrogenase; mitochondrial; EC 1.5.99.2; M2GD; ME2GLYDH
Categories	Primary Antibodies
Clonality	polyclonal
Description	This gene encodes an enzyme involved in the catabolism of choline, catalyzing the oxidative demethylation of dimethylglycine to form sarcosine. The enzyme is found as a monomer in the mitochondrial matrix, and uses flavin adenine dinucleotide and folate as cofactors. Mutation in this gene causes dimethylglycine dehydrogenase deficiency, characterized by a fishlike body odor, chronic muscle fatigue, and elevated levels of the muscle form of creatine kinase in serum. Alternative splicing results in multiple transcript variants.Binzak B.A., Mol. Genet. Metab. 69:181-187(2000). Binzak B.A., Am. J. Hum. Genet. 68:839-847(2001).Moolenaar S.H., Clin. Chem. 45:459-464(1999)
Host	Rabbit
Immunogen	Synthesized peptide derived from C-terminal of Human DMGDH.
Involvement In Disease	DMGDH deficiency (DMGDHD)
Raised In	Rabbit
Reactivity	Human
Regulatory	RUO
Relevance	This gene encodes an enzyme involved in the catabolism of choline, catalyzing the oxidative demethylation of dimethylglycine to form sarcosine. The enzyme is found as a monomer in the mitochondrial matrix, and uses flavin adenine dinucleotide and folate as cofactors. Mutation in this gene causes dimethylglycine dehydrogenase deficiency, characterized by a fishlike body odor, chronic muscle fatigue, and elevated levels of the muscle form of creatine kinase in serum. Alternative splicing results in multiple transcript variants. Binzak B.A., Mol. Genet. Metab. 69:181-187(2000). Binzak B.A., Am. J. Hum. Genet. 68:839-847(2001). Moolenaar S.H., Clin. Chem. 45:459-464(1999)
Species	Homo Sapiens (Human)
Specificity	The antibody detects endogenous levels of total DMGDH protein.
Subcellular Location	Mitochondrion

Uniprot	Q9UI17
Database Links	HGNC:24475 OMIM:605849 KEGG:hsa:29958 STRING:9606.ENSP00000255189 UniGene:Hs.655653
Function	Catalyzes the demethylation of N,N-dimethylglycine to sarcosine. Also has activity with sarcosine in vitro.
Protein Families	GcvT family

Buffer	Rabbit IgG in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
Form	Rabbit IgG in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
Format	liquid
Purification	The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Purity	The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Storage	Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.

Applications

ELISA, WB

Description

ELISA ; WB, WB ; ELISA

Dilution

WB	1:500-1:3000

Tested Application

ELISA,WB;WB:1:500-1:3000

Datasheet
ELISA Protocol
Western Blotting(WB) Protocol

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SKU	Supplier No.	Size	Your price	Quantity
BHA10558379	CSB-PA699345	100ul	$297		Add to Cart

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