Rabbit GCDH Polyclonal Antibody | anti-GCDH antibody

GCDH Antibody

Cat. Num. AAA9611366

• Gene Names: GCDH; GCD; ACAD5
• Reactivity: Human, Mouse, Rat; Predicted: Pig, Bovine, Horse, Sheep, Rabbit, Dog, Chicken, Xenopus
• Applications: Western Blot
• Purity: The antiserum was purified by peptide affinity chromatography using SulfoLink Coupling Resin (Thermo Fisher Scientific).
• Synonyms: GCDH; Polyclonal Antibody; GCDH Antibody; ACAD5; EC 1.3.99.7; GCD; Gcdh; GCDH_HUMAN; Glutaryl CoA dehydrogenase; mitochondrial; Glutaryl Coenzyme A dehydrogenase; Glutaryl-CoA dehydrogenase; MS781; anti-GCDH antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat; Predicted: Pig, Bovine, Horse, Sheep, Rabbit, Dog, Chicken, Xenopus
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: GCDH Antibody detects endogenous levels of GCDH.
• Purity/Purification: The antiserum was purified by peptide affinity chromatography using SulfoLink Coupling Resin (Thermo Fisher Scientific).
• Form/Format: Liquid; Phosphate buffered saline, pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
• Concentration: 1mg/ml (varies by lot)
• Sequence Length: 438

• Applicable Applications for anti-GCDH antibody: Western Blot (WB)
• Application Notes: WB: 1:500-1:2000
• Immunogen: A synthesized peptide derived from human GCDH.
• Tissue Specificity: Isoform Long and isoform Short are expressed in fibroblasts and liver.
• Fragment: Fab Fragment
• Conjugate: Unconjugated
• Subcellular Location: Mitochondrion
• Preparation and Storage: Store at -20 degree C. Stable for 12 months from date of receipt.

Western Blot (WB)

(Western blot analysis of extracts from L02 cells, using GCDH Antibody. The lane on the left was treated with blocking peptide.)

Related Product Information for anti-GCDH antibody

Description: Catalyzes the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO2 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.

Subunit Structure: Homotetramer.

Similarity: Belongs to the acyl-CoA dehydrogenase family.

NCBI and Uniprot Product Information

• NCBI GI #: 4503943
• NCBI GeneID: 2639
• NCBI Accession #: NP_000150.1
• NCBI GenBank Nucleotide #: NP_000150.1
• UniProt Accession #: Q92947
• Molecular Weight: Observed: 48 kDa; Predicted: 49 kDa
• 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: Mitochondrial; Lipid Metabolism - fatty acid; Amino Acid Metabolism - lysine degradation; Oxidoreductase; Amino Acid Metabolism - tryptophan; EC 1.3.8.6

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