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ACAD8, Polyclonal Antibody

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產(chǎn)品名稱: ACAD8, Polyclonal Antibody
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ACAD8, Polyclonal Antibody


ACAD8, Polyclonal Antibody  的詳細(xì)介紹
Product Name

ACAD8, Polyclonal Antibody

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.
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OMIM
604773
Clonality
Polyclonal
Host
Rabbit
Species Reactivity
Human
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
Target Name
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.
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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
anti-ACAD8 antibody Testing Data image
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NCBI/Uniprot data below describe general gene information for ACAD8. It may not necessarily be applicable to this product.
NCBI GI #
7656849
NCBI GeneID
27034
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
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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
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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]
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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)
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Precautions
All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
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.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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