Product Name
Acyl-Coenzyme A Dehydrogenase 8 (ACAD8), Recombinant Protein
Popular Item
Full Product Name
Recombinant Human Acyl-Coenzyme A Dehydrogenase 8
Product Synonym Names
ACAD8 Human; Acyl-Coenzyme A Dehydrogenase 8 Human Recombinant; Acyl-CoA dehydrogenase family member 8 mitochondrial; ACAD-8; Isobutyryl-CoA dehydrogenase; Activator-recruited cofactor 42 kDa component; ARC42; FLJ22590
Product Gene Name
ACAD8 recombinant protein
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Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Sequence
MGSSHHH HHH SSGLVPRGSH MGSLVQ TGHR SLTSCIDPSM GLNEEQKEFQ KVAFDFAARE MAPNMAEWDQ KELFPVDVMR KAAQLGFGGV YIQTDVGGSG LSRLDTSVIF EALATGCTST TAYISIHNMC AWMIDSFGNE EQRHKFCPPL CTMEKFASYC LTEPGSGSDA ASLLTSAKKQ GDHYILNGSK AFISGAGESD IYVVMCRTGG PGPKGISCIV VEKGTPGLSF GKKEKKVGWN SQPTRAVIFE DCAVPVANRI GSEGQGFLIA VRGLNGGRIN IASCSLGAAH ASVILTRDHL NVRKQFGEPL ASNQYLQFTL ADMATRLVAA RLMVRNAAVA LQEERKDAVA LCSMAKLFAT DECFAICNQA LQMHGGYGYL KDYAVQQYVR DSRVHQILEG SNEVMRILIS RSLLQE
3D Structure
ModBase 3D Structure for Q9UKU7
Purity/Purification
Greater than 95% as determined by SDS-PAGE.
Form/Format
The ACAD8 solution contains 20mM Tris-HCl buffer (pH 8.0), 0.15M NaCl, 1mM DTT and 30% glycerol.
Sterile Filtered clear solution.
Preparation and Storage
Store at 4 degree C if entire vial will be used within 2-4 weeks.?Store, frozen at -20 degree C for longer periods of time.?For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).Avoid multiple freeze-thaw cycles.
Other Notes
Small volumes of ACAD8 recombinant protein 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
ACAD8 recombinant protein
Description: ACAD8 Human Recombinant produced in E Coli is a single, non-glycosylated polypeptide chain containing 416 amino acids (23-415) and having a molecular mass of 45.1kDa.ACAD8 is fused to a 23 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques.
Product Categories/Family for ACAD8 recombinant protein
ENZYMES; Enzymes; Dehydrogenase
NCBI/Uniprot data below describe general gene information for ACAD8. It may not necessarily be applicable to this product.
NCBI Accession #
NP_055199.1
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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; activator-recruited cofactor 42 kDa component; acyl-Coenzyme A dehydrogenase family, member 8
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: 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; transcription, DNA-dependent; regulation of transcription, DNA-dependent; lipid metabolic process; branched chain family amino acid catabolic 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
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.
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