Recombinant Human D-2-hydroxyglutarate dehydrogenase, mitochondrial (D2HGDH), Homo sapiens (Human), E.coli, N-terminal 6xHis-SUMO-tagged, 70.8kDa, purity >90% as determined by SDS-PAGE.
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Recombinant Human D-2-hydroxyglutarate dehydrogenase, mitochondrial (D2HGDH)

Recombinant Human D-2-hydroxyglutarate dehydrogenase, mitochondrial (D2HGDH) — Catalyzes the oxidation of D-2-hydroxyglutarate (D-2-HG) to alpha-ketoglutarate. Purity >90%.

SKU: BCRECP-000473 Categories: ,
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Product Specifications

Product SkuBCRECP-000473
Product DescriptionRecombinant Human D-2-hydroxyglutarate dehydrogenase, mitochondrial (D2HGDH) Protein is expressed from E.coli with N-terminal 6xHis-SUMO-tagged. It contains 14-521aa. [Accession | Q8N465].
Uniprot No.Q8N465
Gene NamesD2HGDH
PurityGreater than 90% as determined by SDS-PAGE.
Expression SystemE.coli
Expression Region14-521aa
SpeciesHomo sapiens (Human)
Tag InfoN-terminal 6xHis-SUMO-tagged
Molecular weight70.8kDa
ActivityPlease contact us to obtain bioactivity data.
BufferIf the delivery form is liquid, the default storage buffer is Tris/PBS-based buffer, 5%-50% glycerol. If the delivery form is lyophilized powder, the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose.
StorageStore at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
Research AreasMetabolism

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.

Biological Context

Subcellular Location: Mitochondrion
Disease Association: D-2-hydroxyglutaric aciduria 1 (D2HGA1) : A rare recessive neurometabolic disorder causing developmental delay, epilepsy, hypotonia, and dysmorphic features. Both a mild and a severe phenotype exist. The severe phenotype is homogeneous and is characterized by early infantile-onset epileptic encephalopathy and cardiomyopathy. The mild phenotype has a more variable clinical presentation. Diagnosis is based on the presence of an excess of D-2-hydroxyglutaric acid in the urine. [The disease is caused by variants affecting the gene represented in this entry]

Product Specifications

Recombinant Human D-2-hydroxyglutarate dehydrogenase, mitochondrial (D2HGDH) is a recombinant protein from Homo sapiens (Human), expressed in E.coli, covering amino acids 14-521aa, with N-terminal 6xHis-SUMO-tagged tag, molecular weight 70.8kDa, purity Greater than 90% as determined by SDS-PAGE.. Suitable for ELISA and Western Blot applications.

Recommended applications depend on the target, product format, and product-specific validation data. Refer to the product datasheet or Certificate of Analysis (CoA), or contact BioCrest Sci for application guidance.

Biological Activity: Please contact us to obtain bioactivity data.

Storage: Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.

Shipping & Lead Time: Availability, shipping conditions, and estimated lead time are confirmed in the formal quotation or order confirmation based on the product format, destination, and order requirements.

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Protein Biology

Function

Catalyzes the oxidation of D-2-hydroxyglutarate (D-2-HG) to alpha-ketoglutarate (PubMed:15070399, PubMed:15609246, PubMed:16037974, PubMed:20020533, PubMed:33431826). Also catalyzes the oxidation of other D-2-hydroxyacids, such as D-malate (D-MAL) and D-lactate (D-LAC) (PubMed:33431826). Exhibits high activities towards D-2-HG and D-MAL but a very weak activity towards D-LAC (PubMed:33431826)

Subcellular Location

Mitochondrion

Disease Association

D-2-hydroxyglutaric aciduria 1 (D2HGA1) : A rare recessive neurometabolic disorder causing developmental delay, epilepsy, hypotonia, and dysmorphic features. Both a mild and a severe phenotype exist. The severe phenotype is homogeneous and is characterized by early infantile-onset epileptic encephalopathy and cardiomyopathy. The mild phenotype has a more variable clinical presentation. Diagnosis is based on the presence of an excess of D-2-hydroxyglutaric acid in the urine. [The disease is caused by variants affecting the gene represented in this entry]

Gene: D2HGDH  |  Organism: Homo sapiens
Key Publications

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How do I order or inquire about this product?

Submit the Online Inquiry form with your required quantity and specifications, or email sales@biocrestsci.com. Available sizes, pricing, stock status, documentation, and estimated lead time are confirmed in the formal quotation.

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Availability, dispatch timing, shipping conditions, and delivery estimates depend on the product format, destination, and order requirements. These details are confirmed in the formal quotation or order confirmation.

How should this product be stored?

Follow the storage and handling instructions in the product-specific datasheet and Certificate of Analysis (CoA). Requirements may vary by product form and formulation; contact BioCrest Sci if product-specific guidance is needed.

What quality controls are performed on your products?

Quality-control data such as identity, purity, endotoxin level, or biological activity are provided only when specified on this product page, the datasheet, or Certificate of Analysis (CoA). Biological activity is not implied unless explicitly stated.

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