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


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Summary

Host Animal
Rabbit
Specificity
Human, Mouse, Rat
Clone
CBYCD-321
Antibody Isotype
IgG
Application
WB

Basic Information

Specificity
Human, Mouse, Rat
Antibody Isotype
IgG
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Storage
Store at 4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
Dimethylglycine Dehydrogenase
Introduction
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.
Entrez Gene ID
Human29958
Mouse74129
Rat245961
UniProt ID
HumanQ9UI17
MouseQ9DBT9
RatQ63342
Alternative Names
Dimethylglycine Dehydrogenase; ME2GLYDH; Dimethylglycine Dehydrogenase, Mitochondrial; EC 1.5.8.4; DMGDHD;
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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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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