ACAD-8; FLJ22590

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

Polyclonal

Rabbit

Purified by antigen-affinity chromatography.

Supplied in 0.1M Tris-buffered saline with 10% Glycerol (pH7.0). 0.01% Thimerosal was added as a preservative.

Store at -20 degree C for long term preservation (recommended). Store at 4 degree C for short term use.

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.

Isobutyryl-CoA dehydrogenase (EC 1.3.99), encoded by the ACAD8 gene, catalyzes the third step of the degradation of the branched chain amino acid valine (Nguyen et al., 2002 [PubMed 12359132]).[supplied by OMIM]

Total protein Ab

Western Blot (WB)

Western blotting: 1:500-1:3000

Sample (30 ug of whole cell lysate)A: 293T10% SDS PAGEPrimary antibody diluted at 1: 500

38,359 Da

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

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

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