Full Product Name
ACAD8 Antibody; FITC conjugated
Product Synonym Names
Isobutyryl-CoA dehydrogenase; mitochondrial; Activator-recruited cofactor 42 kDa component; ARC42; Acyl-CoA dehydrogenase family member 8; ACAD-8; ACAD8; ARC42; IBD
Product Gene Name
anti-ACAD8 antibody
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
3D Structure
ModBase 3D Structure for Q9UKU7
Purity/Purification
>95%,Protein G purified
Immunogen
Recombinant human Isobutyryl-CoA dehydrogenase, mitochondrial protein
Storage Buffer
Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
Santa Cruz Alternative
Potential replacement for Santa Cruz Biotechnology antibody catalog# sc-66708 / sc-66709 / sc-66706 / sc-135147
Preparation and Storage
Shipped at 4 degree C. Upon delivery, aliquot and store at -20 degree C or -80 degree C.
ISO Certification
Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.
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
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.
Applications Tested/Suitable for anti-ACAD8 antibody
ELISA (EIA)
NCBI/Uniprot data below describe general gene information for ACAD8. It may not necessarily be applicable to this product.
NCBI Accession #
NP_055199.1
[Other Products]
NCBI GenBank Nucleotide #
NM_014384.2
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UniProt Primary Accession #
Q9UKU7
[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: EC 1.3.99.-; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Oxidoreductase; Mitochondrial
Chromosomal Location of Human Ortholog: 11q25
Cellular Component: mitochondrial matrix; mitochondrion
Molecular Function: acyl-CoA binding; acyl-CoA dehydrogenase activity; electron carrier activity; FAD binding
Biological Process: branched chain family amino acid catabolic process; fatty acid beta-oxidation using acyl-CoA dehydrogenase; lipid homeostasis; lipid metabolic process; regulation of transcription, DNA-dependent; transcription, DNA-dependent; valine catabolic process
Disease: Isobutyryl-coa Dehydrogenase Deficiency
Research Articles on ACAD8
1. we discovered a novel c.1156_1158delCAG mutation in ACAD8 in patients with isobutyryl-CoA dehydrogenase deficiency , and investigated the mutation spectrum of ACAD8.
Precautions
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