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.

SDS-PAGE: Single band at expected molecular weight confirming purity.

ELISA: Suitable as coating antigen or detection standard.

Western Blot: Compatible with standard Western Blot protocols.

Protein Interaction: Validated for SPR (Surface Plasmon Resonance) and BLI (Bio-Layer Interferometry) studies.

Shipping: Shipped at ambient temperature. Lyophilized protein is stable during transit.

Storage: Store lyophilized protein at -20°C to -80°C. Reconstituted protein should be aliquoted and stored at -80°C. Avoid repeated freeze-thaw cycles.

Shelf Life: 12 months from date of receipt when stored as recommended.

Shipping Time: Orders placed before 2 PM EST ship same day. International orders typically deliver within 5-10 business days.

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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

Frequently Asked Questions

How do I order or inquire about this product?

Fill out the Online Inquiry form with your required quantity and specifications. You can also email sales@biocrestsci.com. Our team typically responds within 4 business hours with a quote and availability confirmation.

What is the shipping and delivery time?

Orders placed before 2 PM EST ship the same day. Domestic (US) delivery typically takes 2-3 business days. International orders deliver within 5-10 business days. All products are shipped at ambient temperature with appropriate packaging to ensure stability.

How should I store this recombinant protein?

Lyophilized proteins should be stored at -20°C to -80°C upon receipt. After reconstitution, aliquot and store at -80°C. Avoid repeated freeze-thaw cycles. Shelf life is 12 months from date of receipt when stored as recommended.

What quality controls are performed on your products?

Each product undergoes SDS-PAGE purity analysis (typically >85-95%), endotoxin testing, and bioactivity validation. Products are validated for ELISA, Western Blot, and SPR/BLI applications as specified on this product page. A Certificate of Analysis (CoA) is available upon request.

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