Rabbit anti-Human, Mouse ACAD8 Polyclonal Antibody | anti-ACAD8 antibody

ACAD8, ID (ACAD8, ARC42, IBD, Isobutyryl-CoA dehydrogenase, mitochondrial, Activator-recruited cofactor 42kD component, Acyl-CoA dehydrogenase family member 8) (APC)

Cat. Num. AAA6270761

• Gene Names: ACAD8; ARC42; ACAD-8
• Reactivity: Human, Mouse
• Applications: Western Blot
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: ACAD8; Polyclonal Antibody; ID (ACAD8; ARC42; IBD; Isobutyryl-CoA dehydrogenase; mitochondrial; Activator-recruited cofactor 42kD component; Acyl-CoA dehydrogenase family member 8) (APC); Acyl-CoA dehydrogenase family member 8; anti-ACAD8 antibody

• Host: Rabbit
• Reactivity: Human, Mouse
• 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 Allophycocyanin (APC).

• Applicable Applications for anti-ACAD8 antibody: Western Blot (WB), FLISA
• Application Notes: WB: 1:100-500; FLISA: 1:1,000; Applications are based on unconjugated antibody.
• Immunogen: ACAD8 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 125~154 amino acids from the Central region of human ACAD8.
• Conjugate: APC
• Note: Preservative Free
• Special Handling: Light sensitive
• Preparation and Storage: Store product at 4 degree C in the dark. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: APC conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

Related Product Information for anti-ACAD8 antibody

ACAD8 is a member of the acyl-CoA dehydrogenase family of enzymes that catalyze the dehydrogenation of acyl-CoA derivatives in the metabolism of fatty acids or branch chained amino acids. This protein is a mitochondrial enzyme that functions in catabolism of the branched-chain amino acid valine.

Product Categories/Family for anti-ACAD8 antibody

Antibodies; Mitochondrial Proteins

References

Wollmer, M.A., et al. Neurogenetics 8(3):179-188(2007). Ma, J., et al. Atherosclerosis 191(1):63-72(2007). Battaile, K.P., et al. J. Biol. Chem. 279(16):16526-16534(2004).

NCBI and Uniprot Product Information

• NCBI GI #: 7656849
• NCBI GeneID: 27034
• NCBI Accession #: NP_055199.1
• NCBI GenBank Nucleotide #: NM_014384.2
• UniProt Accession #: Q9UKU7; Q6ZWP6; Q9BUS8; B7Z5W4
• Molecular Weight: 38,359 Da
• NCBI Official Full Name: isobutyryl-CoA dehydrogenase, mitochondrial
• NCBI Official Synonym Full Names: acyl-CoA dehydrogenase family, member 8
• NCBI Official Symbol: ACAD8
• NCBI Official Synonym Symbols: ARC42; ACAD-8
• NCBI Protein Information: isobutyryl-CoA dehydrogenase, mitochondrial; activator-recruited cofactor 42 kDa component; acyl-Coenzyme A dehydrogenase family, member 8
• UniProt Protein Name: Isobutyryl-CoA dehydrogenase, mitochondrial
• Protein Family: Isobutyryl-CoA dehydrogenase
• UniProt Gene Name: ACAD8
• UniProt Synonym Gene Names: ARC42; IBD; ARC42; ACAD-8
• UniProt Entry Name: ACAD8_HUMAN

NCBI Description

This gene encodes a member of the acyl-CoA dehydrogenase family of enzymes that catalyze the dehydrogenation of acyl-CoA derivatives in the metabolism of fatty acids or branch chained amino acids. The encoded protein is a mitochondrial enzyme that functions in catabolism of the branched-chain amino acid valine. Defects in this gene are the cause of isobutyryl-CoA dehydrogenase deficiency.[provided by RefSeq, Nov 2009]

Uniprot Description

ACAD8: Has very high activity toward isobutyryl-CoA. Is an isobutyryl-CoA dehydrogenase that functions in valine catabolism. Plays a role in transcriptional coactivation within the ARC complex. Defects in ACAD8 are the cause of isobutyryl-CoA dehydrogenase deficiency (IBDD). The symptoms of IBDD generally appear until late in infancy or in childhood and can include poor feeding and growth (failure to thrive), a weakened and enlarged heart (dilated cardiomyopathy), seizures, and low numbers of red blood cells (anemia). Belongs to the acyl-CoA dehydrogenase family.

Protein type: Oxidoreductase; EC 1.3.99.-; Mitochondrial; Amino Acid Metabolism - valine, leucine and isoleucine degradation

Chromosomal Location of Human Ortholog: 11q25

Cellular Component: mitochondrial matrix

Molecular Function: acyl-CoA dehydrogenase activity; FAD binding

Biological Process: valine catabolic process; transcription, DNA-dependent; regulation of transcription, DNA-dependent; lipid metabolic process; branched chain family amino acid catabolic process

Disease: Isobutyryl-coa Dehydrogenase Deficiency