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ACADSB blocking peptide

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Catalog # MBS9229382
Unit / Price
  0.1 mg  /  $155 +1 FREE 8GB USB
ACADSB blocking peptide
Product Name

ACADSB, Blocking Peptide

Also Known As

ACADSB Blocking Peptide (Center)

Product Synonym Names
Short/branched chain specific acyl-CoA dehydrogenase; mitochondrial; SBCAD; 2-methyl branched chain acyl-CoA dehydrogenase; 2-MEBCAD; 2-methylbutyryl-coenzyme A dehydrogenase; 2-methylbutyryl-CoA dehydrogenase; ACADSB
Antibody/Peptide Pairs
ACADSB peptide (MBS9229382) is used for blocking the activity of ACADSB antibody (MBS9208091)
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Sequence Length
432
OMIM
600301
3D Structure
ModBase 3D Structure for P45954
Specificity
The synthetic peptide sequence is selected from aa 239-253 of HUMAN ACADSB
Form/Format
Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.
Cellular Location
Mitochondrion matrix.
Tissue Location
Ubiquitous.
Preparation and Storage
Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C.
Other Notes
Small volumes of ACADSB blocking peptide 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
ACADSB blocking peptide
Has greatest activity toward short branched chain acyl- CoA derivative such as (s)-2-methylbutyryl-CoA, isobutyryl-CoA, and 2-methylhexanoyl-CoA as well as toward short straight chain acyl-CoAs such as butyryl-CoA and hexanoyl-CoA. Can use valproyl- CoA as substrate and may play a role in controlling the metabolic flux of valproic acid in the development of toxicity of this agent.
NCBI/Uniprot data below describe general gene information for ACADSB. It may not necessarily be applicable to this product.
NCBI GI #
NCBI GeneID
NCBI Accession #
UniProt Primary Accession #
UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
36,025 Da
NCBI Official Full Name
Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
NCBI Official Synonym Full Names
acyl-CoA dehydrogenase, short/branched chain
NCBI Official Symbol
ACADSB  [Similar Products]
NCBI Official Synonym Symbols
ACAD7; SBCAD; 2-MEBCAD
  [Similar Products]
NCBI Protein Information
short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
UniProt Protein Name
Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
UniProt Synonym Protein Names
2-methyl branched chain acyl-CoA dehydrogenase; 2-MEBCAD; 2-methylbutyryl-coenzyme A dehydrogenase; 2-methylbutyryl-CoA dehydrogenase
UniProt Gene Name
ACADSB  [Similar Products]
UniProt Synonym Gene Names
SBCAD; 2-MEBCAD; 2-methylbutyryl-CoA dehydrogenase  [Similar Products]
UniProt Entry Name
ACDSB_HUMAN
NCBI Summary for ACADSB
Short/branched chain acyl-CoA dehydrogenase(ACADSB) is 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. Substrate specificity is the primary characteristic used to define members of this gene family. The ACADSB gene product has the greatest activity towards the short branched chain acyl-CoA derivative, (S)-2-methylbutyryl-CoA, but also reacts significantly with other 2-methyl branched chain substrates and with short straight chain acyl-CoAs. The cDNA encodes for a mitochondrial precursor protein which is cleaved upon mitochondrial import and predicted to yield a mature peptide of approximately 43.7-KDa. [provided by RefSeq, Jul 2008]
UniProt Comments for ACADSB
ACADSB: Has greatest activity toward short branched chain acyl- CoA derivative such as (s)-2-methylbutyryl-CoA, isobutyryl-CoA, and 2-methylhexanoyl-CoA as well as toward short straight chain acyl-CoAs such as butyryl-CoA and hexanoyl-CoA. Can use valproyl- CoA as substrate and may play a role in controlling the metabolic flux of valproic acid in the development of toxicity of this agent. Defects in ACADSB are the cause of short/branched-chain acyl-CoA dehydrogenase deficiency (SBCADD); also known as 2-methylbutyryl-CoA dehydrogenase deficiency or 2- methylbutyryl glycinuria. SBCADD is an autosomal recessive disorder and consists of a defect in catabolism of L-isoleucine which is characterized by an increase of 2-methylbutyrylglycine and 2-methylbutyrylcarnitine in blood and urine. Affected individuals have seizures and psychomotor delay as the main clinical features. Belongs to the acyl-CoA dehydrogenase family.

Protein type: Lipid Metabolism - fatty acid; Mitochondrial; Oxidoreductase; Amino Acid Metabolism - valine, leucine and isoleucine degradation; EC 1.3.8.5

Chromosomal Location of Human Ortholog: 10q26.13

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; fatty acid metabolic process; lipid homeostasis

Disease: 2-methylbutyryl-coa Dehydrogenase Deficiency
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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