Rabbit anti-Human GCDH Polyclonal Antibody | anti-GCDH antibody

GCDH, CT (GCDH, Glutaryl-CoA dehydrogenase, mitochondrial) (Biotin)

Cat. Num. AAA6301579

• Gene Names: GCDH; GCD; ACAD5
• Reactivity: Human
• Applications: Immunohistochemistry, ELISA, Western Blot
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: GCDH; Polyclonal Antibody; CT (GCDH; Glutaryl-CoA dehydrogenase; mitochondrial) (Biotin); mitochondrial; anti-GCDH antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Biotin.

• Applicable Applications for anti-GCDH antibody: Immunohistochemistry (IHC), ELISA (EIA), Western Blot (WB)
• Application Notes: IHC: 1:10-50; ELISA: 1:1,000; WB: 1:100-500; Applications are based on unconjugated antibody.
• Immunogen: GCDH antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 335-365 amino acids from the C-terminal region of human GCDH.
• Conjugate: Biotin
• Note: Preservative Free
• Preparation and Storage: Store product at 4 degree C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20 degree C. Aliquots are stable at -20 degree C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(Western blot analysis of GCDH (arrow) using rabbit polyclonal GCDH Antibody (C-term). 293 cell lysates (2 ug/lane) either nontransfected (Lane 1) or transiently transfected (Lane 2) with the GCDH gene.)

Immunohistochemistry (IHC)

(GCDH Antibody (C-term) immunohistochemistry analysis in formalin fixed and paraffin embedded human liver tissue followed by peroxidase conjugation of the secondary antibody and DAB staining.This data demonstrates the use of GCDH Antibody (C-term) for immunohistochemistry. Clinical relevance has not been evaluated.)

Related Product Information for anti-GCDH antibody

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. Alternatively spliced transcript variants encoding different isoforms have been identified.

Product Categories/Family for anti-GCDH antibody

Antibodies; Mitochondrial Proteins

References

Bailey, S.D., et al. Diabetes Care 33(10):2250-2253(2010). Park, J.D., et al. J. Korean Med. Sci. 25(6):957-960(2010). Strauss, K.A., et al. Brain 133 (PT 1), 76-92 (2010): Talmud, P.J., et al. Am. J. Hum. Genet. 85(5):628-642(2009). Ganesh, S.K., et al. Nat. Genet. 41(11):1191-1198(2009).

NCBI and Uniprot Product Information

• NCBI GI #: 4503943
• NCBI GeneID: 2639
• NCBI Accession #: NP_000150.1; NP_039663.1
• 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; 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: 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