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Recombinant Human D-2-hydroxyglutarate dehydrogenase, mitochondrial (D2HGDH)

ACP13707

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Specifications

Cat.No	ACP13707	Target Name	d2hgdh
Target Synonyms	D2HGDH; D2HGDD-2-hydroxyglutarate dehydrogenase; mitochondrial; EC 1.1.99.-	Form	Lyophilized powder
Expression System	Custom Production. Please inquire and provide the desire expression system.	Expression Range	14-521
Protein Length	Full Length of Mature Protein	Purity	>85% (SDS-PAGE)
Storage Buffer	5%-50% glycerol. Lyophilized powder form: the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, Liquid form: default storage buffer is Tris/PBS-based buffer, pH 8.0.

Immunogen Information

Target Species	Human	Uniprot ID	Q8N465

Background Information

Uniprot Id

Q8N465
Target Species

Human
Target Name

D2HGDH
Target Full Name

D-2-hydroxyglutarate dehydrogenase, mitochondrial
Target Function

Catalyzes the oxidation of D-2-hydroxyglutarate (D-2-HG) to alpha-ketoglutarate. Also catalyzes the oxidation of other D-2-hydroxyacids, such as D-malate (D-MAL) and D-lactate (D-LAC). Exhibits high activities towards D-2-HG and D-MAL but a very weak activity towards D-LAC.
Target Involvement

D-2-hydroxyglutaric aciduria 1 (D2HGA1)
Target Subcellular Location

Mitochondrion.
Target Protein Families

FAD-binding oxidoreductase/transferase type 4 family
Target Research Area

Metabolism
Target Synonyms

D2HGDH; D2HGDD-2-hydroxyglutarate dehydrogenase; mitochondrial; EC 1.1.99.-
Target Background

This gene encodes D-2hydroxyglutarate dehydrogenase, a mitochondrial enzyme belonging to the FAD-binding oxidoreductase/transferase type 4 family. This enzyme, which is most active in liver and kidney but also active in heart and brain, converts D-2-hydroxyglutarate to 2-ketoglutarate. Mutations in this gene are present in D-2-hydroxyglutaric aciduria, a rare recessive neurometabolic disorder causing developmental delay, epilepsy, hypotonia, and dysmorphic features.

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Recombinant Human D-2-hydroxyglutarate dehydrogenase, mitochondrial (D2HGDH)

Specifications

Immunogen Information

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