Mouse Glutaryl-CoA dehydrogenase, mitochondrial ELISA Kit | Gcdh elisa kit

Mouse Glutaryl-CoA dehydrogenase, mitochondrial ELISA Kit

Cat. Num. AAA2888182

• Gene Names: GCDH; GCD; ACAD5
• Reactivity: Mouse
• Synonyms: Glutaryl-CoA dehydrogenase; mitochondrial; Mouse Glutaryl-CoA dehydrogenase; mitochondrial ELISA Kit; Gcdh elisa kit

• Reactivity: Mouse
• Sequence Length: 438

• Assay Type: Sandwich
• Detection Range: 0.625-40 ng/mL
• Sensitivity: 0.321 ng/mL
• Intra-Assay CV: <=5.4%
• Inter-Assay CV: <=8.0%
• Recovery: 89%

Typical Testing Data/Standard Curve (for reference only)

NCBI and Uniprot Product Information

• NCBI GI #: 4503943
• NCBI GeneID: 2639
• NCBI Accession #: NP_000150.1
• NCBI GenBank Nucleotide #: NM_000159.3
• UniProt Accession #: Q92947; O14719; A8K2Z2
• Molecular Weight: 47,355 Da
• 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

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: 19p13.13

Cellular Component: mitochondrial matrix; mitochondrion

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

Biological Process: fatty acid beta-oxidation using acyl-CoA dehydrogenase; fatty-acyl-CoA biosynthetic process; lysine catabolic process; tryptophan metabolic process

Disease: Glutaric Acidemia I