Molecular weight (DA)
97kD
Immunogen
The antiserum was produced against synthesized peptide derived from human DMGDH. AA range:817-866
Specificity
DMGDH Polyclonal Antibody detects endogenous levels of DMGDH protein.
Formulation
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Dilution rate
WB: 1/500 - 1/2000. IHC: 1/100 - 1/300. ELISA: 1/20000.. IF 1:50-200
Purification process (Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
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],
Function
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.,
Protein name
Dimethylglycine dehydrogenase mitochondrial
Other name
DMGDH; Dimethylglycine dehydrogenase; mitochondrial; ME2GLYDH
Fields
>>Glycine, serine and threonine metabolism;>>Metabolic pathways
Human protein sequence Database
Q9UI17
Mouse protein sequence database
Q9DBT9
Rat protein sequence database
Cellular localization
Mitochondrion.
Tissue expression
Kidney,Trachea,
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