Anti -ACAD8, NT (Acyl-Coenzyme A Dehydrogenase 8)

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

Chromosome: 11; NC_000011.9 (134123434..134135749). Location: 11q25

Polyclonal

IgY

Chicken

Recognizes human ACAD8.

Affinity Purified
Purified by immunoaffinity chromatography.

Supplied as a liquid in PBS, pH 7.2, 0.02% sodium azide.

May be stored at 4 degree C for short-term only. For long-term storage, store at -20 degree C. Aliquots are stable for at least 12 months at -20 degree C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.

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.

Antibodies; Abs to Coenzyme A

ELISA (EL/EIA), Western Blot (WB)

Suitable for use in ELISA and Western Blot.
Dilution: Western Blot: 1:1000-1:2000

45,070 Da[Similar Products]

acyl-CoA dehydrogenase family, member 8

isobutyryl-CoA dehydrogenase, mitochondrial; OTTHUMP00000235422; OTTHUMP00000235425; activator-recruited cofactor 42 kDa component; acyl-Coenzyme A dehydrogenase family, member 8

Isobutyryl-CoA dehydrogenase, mitochondrial

ARC42; IBD??[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.

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

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; branched chain family amino acid catabolic process; lipid metabolic process

Disease: Isobutyryl-coa Dehydrogenase Deficiency

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