Glutaryl Coenzyme A Dehydrogenase Recombinant Protein | GCDH recombinant protein

Recombinant Glutaryl Coenzyme A Dehydrogenase (GCDH)

Cat. Num. AAA2034231

• Gene Names: GCDH; GCD; ACAD5
• Reactivity: Mouse
• Applications: SDS-Page, Western Blot, ELISA, Immunoprecipitation
• Purity: >95%
• Synonyms: Glutaryl Coenzyme A Dehydrogenase; Recombinant Glutaryl Coenzyme A Dehydrogenase (GCDH); GCD; ACAD5; Glutaryl-CoA dehydrogenase; mitochondrial; GCDH recombinant protein

• Host: E Coli
• Reactivity: Mouse
• Purity/Purification: >95%
• Sequence Length: 438

• Applicable Applications for GCDH recombinant protein: SDS-PAGE, Western Blot (WB), ELISA (EIA), Immunoprecipitation (IP)
• Fragment: Glu270~Lys438
• Tag: Two N-terminal Tags, His-tag and T7-tag
• Expression System: Prokaryotic Expression
• Preparation and Storage: Storage: Avoid repeated freeze/thaw cycles. Store at 2-8 degree C for one month. Aliquot and store at -80 degree C for 12 months.; Stability Test: The thermal stability is described by the loss rate of the target protein. The loss rate was determined by accelerated thermal degradation test, that is, incubate the protein at 37 degree C for 48h, and no obvious degradation and precipitation were observed. The loss of this protein is less than 5% within the expiration date under appropriate storage condition.

SDS-Page

NCBI and Uniprot Product Information

• NCBI GI #: 4503943
• NCBI GeneID: 2639
• NCBI Accession #: NP_000150.1
• NCBI GenBank Nucleotide #: NM_000159.3
• Molecular Weight: 22.1kDa
• NCBI Official Full Name: glutaryl-CoA dehydrogenase, mitochondrial isoform a
• NCBI Official Synonym Full Names: glutaryl-CoA dehydrogenase
• NCBI Official Symbol: GCDH
• NCBI Official Synonym Symbols: GCD; ACAD5
• NCBI Protein Information: glutaryl-CoA dehydrogenase, mitochondrial
• UniProt Protein Name: Glutaryl-CoA dehydrogenase, mitochondrial
• UniProt Gene Name: GCDH
• UniProt Synonym Gene Names: GCD
• UniProt Entry Name: GCDH_HUMAN

NCBI Description

The protein encoded by this gene belongs to the acyl-CoA dehydrogenase family. It catalyzes the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO(2) in the degradative pathway of L-lysine, L-hydroxylysine, and L-tryptophan metabolism. It uses electron transfer flavoprotein as its electron acceptor. The enzyme exists in the mitochondrial matrix as a homotetramer of 45-kD subunits. Mutations in this gene result in the metabolic disorder glutaric aciduria type 1, which is also known as glutaric acidemia type I. Alternative splicing of this gene results in multiple transcript variants. A related pseudogene has been identified on chromosome 12. [provided by RefSeq, Mar 2013]

Uniprot Description

GCDH: Catalyzes the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO(2) in the degradative pathway of L-lysine, L-hydroxylysine, and L-tryptophan metabolism. It uses electron transfer flavoprotein as its electron acceptor. Isoform Short is inactive. Defects in GCDH are the cause of glutaric aciduria type 1 (GA1). GA1 is an autosomal recessive metabolic disorder characterized by progressive dystonia and athetosis due to gliosis and neuronal loss in the basal ganglia. Belongs to the acyl-CoA dehydrogenase family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Amino Acid Metabolism - lysine degradation; Amino Acid Metabolism - tryptophan; EC 1.3.8.6; Lipid Metabolism - fatty acid; Mitochondrial; Oxidoreductase

Chromosomal Location of Human Ortholog: 19p13.2

Cellular Component: mitochondrial matrix; mitochondrion

Molecular Function: acyl-CoA binding; electron carrier activity; FAD binding; glutaryl-CoA dehydrogenase activity

Biological Process: fatty acid beta-oxidation using acyl-CoA dehydrogenase; lipid homeostasis; lysine catabolic process

Disease: Glutaric Acidemia I