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DMGDH

DMGDH is 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.

Full Name

Dimethylglycine Dehydrogenase

Function

Catalyzes the demethylation of N,N-dimethylglycine to sarcosine. Also has activity with sarcosine in vitro.

Biological Process

Amino-acid betaine catabolic process Source: UniProtKB-UniPathway
Choline catabolic process Source: UniProtKB
Choline metabolic process Source: UniProtKB

Cellular Location

Mitochondrion

Involvement in disease

DMGDH deficiency (DMGDHD):
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.

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Anti-DMGDH antibodies

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Mouse Anti-DMGDH Recombinant Antibody (CBYCD-321) (CBMAB-D1195-YC)

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Target: DMGDH

Host: Mouse

Antibody Isotype: IgG2b, κ

Specificity: Human, Mouse, Rat, Horse, Dog, Cattle

Clone: CBYCD-321

Application*: WB, IP, IF, E

Mouse Anti-DMGDH Recombinant Antibody (1034) (V2LY-0425-LY573)

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Target: DMGDH

Host: Mouse

Antibody Isotype: IgG2a, κ

Specificity: Human, Mouse, Rat

Clone: 1034

Application*: WB, IP, IF, E

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(P): Predicted

* Abbreviations

AActivation
AGAgonist
APApoptosis
BBlocking
BABioassay
BIBioimaging
CImmunohistochemistry-Frozen Sections
CIChromatin Immunoprecipitation
CTCytotoxicity
CSCostimulation
DDepletion
DBDot Blot

EELISA
ECELISA(Cap)
EDELISA(Det)
ESELISpot
EMElectron Microscopy
FFlow Cytometry
FNFunction Assay
GSGel Supershift
IInhibition
IAEnzyme Immunoassay
ICImmunocytochemistry
IDImmunodiffusion
IEImmunoelectrophoresis

IFImmunofluorescence
IGImmunochromatography
IHImmunohistochemistry
IMImmunomicroscopy
IOImmunoassay
IPImmunoprecipitation
ISIntracellular Staining for Flow Cytometry
LALuminex Assay
LFLateral Flow Immunoassay
MMicroarray
MCMass Cytometry/CyTOF
MDMeDIP

MSElectrophoretic Mobility Shift Assay
NNeutralization
PImmunohistologyp-Paraffin Sections
PAPeptide Array
PEPeptide ELISA
PLProximity Ligation Assay
RRadioimmunoassay
SStimulation
SESandwich ELISA
SHIn situ hybridization
TCTissue Culture
WBWestern Blot

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