DMGDH Rabbit Polyclonal Antibody

Description

EnkiLife offers the DMGDH Rabbit Polyclonal Antibody. This antibody reacts with Human,Rat,Mouse species and is suitable for WB,IHC,ICC/IF,ELISA application. Alternative Names of Immunogen: DMGDH; Dimethylglycine dehydrogenase; mitochondrial; ME2GLYDH. The product boasts remarkable high specificity, outstanding reproducibility, and exceptional affinity, making it a reliable and superior choice for your needs. Dilution Ratio: WB 1:500-1:2000,IHC 1:100-1:300,ICC/IF 1:50-1:200,ELISA 1:10000-1:20000 Background: 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. [provided by RefSeq, Jul 2013],catalytic activity:N,N-dimethylglycine + acceptor + H(2)O = sarcosine + formaldehyde + reduced acceptor.,cofactor:Binds 1 FAD covalently per monomer.,disease:Defects in DMGDH are the cause of DMGDH deficiency (DMGDHD) [MIM:605850]. DMGDHD is a disorder characterized by fish odor, muscle fatigue with increased serum creatine kinase. Biochemically it is characterized by an increase of N,N-dimethylglycine (DMG) in serum and urine.,pathway:Amine and polyamine degradation; betaine degradation; sarcosine from betaine: step 2/2.,similarity:Belongs to the gcvT family.,subunit:Monomer., Research Area: Glycine; serine and threonine metabolism;