Full Product Name
ACAD8 antibody
Product Synonym Names
ACAD-8; FLJ22590
Product Gene Name
anti-ACAD8 antibody
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Purity/Purification
Purified by antigen-affinity chromatography.
Form/Format
Supplied in 0.1M Tris-buffered saline with 10% Glycerol (pH7.0). 0.01% Thimerosal was added as a preservative.
Immunogen Type
Recombinant protein
Immunogen Description
Recombinant protein fragment contain a sequence corresponding to a region within amino acids 146 and 411 of Human ACAD8
Preparation and Storage
Store at -20 degree C for long term preservation (recommended). Store at 4 degree C for short term use.
Other Notes
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.
Related Product Information for
anti-ACAD8 antibody
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]
Product Categories/Family for anti-ACAD8 antibody
Total protein Ab
Applications Tested/Suitable for anti-ACAD8 antibody
Western Blot (WB)
Application Notes for anti-ACAD8 antibody
Western blotting: 1:500-1:3000
Testing Data of anti-ACAD8 antibody
Sample (30 ug of whole cell lysate)A: 293T10% SDS PAGEPrimary antibody diluted at 1: 500

NCBI/Uniprot data below describe general gene information for ACAD8. It may not necessarily be applicable to this product.
NCBI Accession #
NP_055199
[Other Products]
NCBI GenBank Nucleotide #
NM_014384.2
[Other Products]
UniProt Secondary Accession #
Q6ZWP6; Q9BUS8; B7Z5W4[Other Products]
UniProt Related Accession #
Q9UKU7[Other Products]
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??[Similar Products]
NCBI Official Synonym Symbols
ARC42; ACAD-8
??[Similar Products]
NCBI Protein Information
isobutyryl-CoA dehydrogenase, mitochondrial
UniProt Protein Name
Isobutyryl-CoA dehydrogenase, mitochondrial
UniProt Synonym Protein Names
Activator-recruited cofactor 42 kDa component; ARC42; Acyl-CoA dehydrogenase family member 8; ACAD-8
Protein Family
Isobutyryl-CoA dehydrogenase
UniProt Gene Name
ACAD8??[Similar Products]
UniProt Synonym Gene Names
ARC42; IBD; ARC42; ACAD-8??[Similar Products]
UniProt Entry Name
ACAD8_HUMAN
NCBI Summary for ACAD8
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 Comments for ACAD8
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
Research Articles on ACAD8
1. Observational study of gene-disease association. (HuGE Navigator)
Precautions
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Disclaimer
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