Human Glutaryl CoA dehydrogenase, mitochondrial (GCDH) ELISA Kit | GCDH elisa kit

Human Glutaryl CoA dehydrogenase, mitochondrial (GCDH) ELISA Kit

Cat. Num. AAA7207976

• Gene Names: GCDH; GCD; ACAD5
• Reactivity: Human
• Synonyms: Glutaryl CoA dehydrogenase; mitochondrial (GCDH); Human Glutaryl CoA dehydrogenase; mitochondrial (GCDH) ELISA Kit; GCDH elisa kit

• Reactivity: Human

• Samples: Serum, Plasma, Cell Culture Supernatants, Body Fluid And Tissue Homogenate
• Assay Type: Quantitative Competitive
• Sensitivity: 0.1 ng/mL.
• Preparation and Storage: Store all reagents at 2-8 degree C.

Typical Testing Data/Standard Curve (for reference only)

Related Product Information for GCDH elisa kit

Intended Uses: This GC ELISA kit is a 1.5 hour solid-phase ELISA designed for the quantitative determination of Rat GC. This ELISA kit for research use only!

Principle of the Assay: GC ELISA kit applies the competitive enzyme immunoassay technique utilizing a polyclonal anti-GC antibody and an GC-HRP conjugate. The assay sample and buffer are incubated together with GC-HRP conjugate in pre-coated plate for one hour. After the incubation period, the wells are decanted and washed five times. The wells are then incubated with a substrate for HRP enzyme. The product of the enzyme-substrate reaction forms a blue colored complex. Finally, a stop solution is added to stop the reaction, which will then turn the solution yellow. The intensity of color is measured spectrophotometrically at 450nm in a microplate reader. The intensity of the color is inversely proportional to the GC concentration since GC from samples and GC-HRP conjugate compete for the anti-GC antibody binding site. Since the number of sites is limited, as more sites are occupied by GC from the sample, fewer sites are left to bind GC-HRP conjugate. A standard curve is plotted relating the intensity of the color (O.D.) to the concentration of standards. The GC concentration in each sample is interpolated from this standard curve.

NCBI and Uniprot Product Information

• NCBI GI #: 7669494
• NCBI GeneID: 2639
• NCBI Accession #: NP_039663.1
• NCBI GenBank Nucleotide #: NM_013976.3
• Molecular Weight: 47,355 Da
• NCBI Official Full Name: glutaryl-CoA dehydrogenase, mitochondrial isoform b
• 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; glutaryl-Coenzyme A dehydrogenase
• 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; Lipid Metabolism - fatty acid; EC 1.3.8.6; Oxidoreductase; Mitochondrial; Amino Acid Metabolism - tryptophan

Chromosomal Location of Human Ortholog: 19p13.2

Cellular Component: mitochondrion; mitochondrial matrix; mitochondrial inner membrane

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

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

Disease: Glutaric Acidemia I