Isobutyryl-CoA dehydrogenase; mitochondrial; 1399-; Activator-recruited cofactor 42 kDa component; ARC42; Acyl-CoA dehydrogenase family member 8; ACAD-8; ACAD8; ARC42; IBD

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit Ig

Rabbit

This ACAD8 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 118-145 amino acids from the Central region of human ACAD8.

Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.

Vial Concentration: 0.5 (lot specific)

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Small volumes of anti-ACAD8 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

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.

Metabolism; Signal Transduction

Western Blot (WB), ELISA (EIA)

WB~~1:1000

Western blot analysis of ACAD8 Antibody (Center) in mouse stomach tissue lysates (35ug/lane). ACAD8 (arrow) was detected using the purified Pab.

45070

acyl-CoA dehydrogenase family, member 8

isobutyryl-CoA dehydrogenase, mitochondrial

Isobutyryl-CoA dehydrogenase, mitochondrial

ARC42; IBD; ARC42; ACAD-8??[Similar Products]

ACAD8_HUMAN

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]

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

Disease: Isobutyryl-coa Dehydrogenase Deficiency

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)

All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.

While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.