Product Name
acyl-Coenzyme A dehydrogenase, short/branched chain (ACADSB), Polyclonal Antibody
Full Product Name
Rabbit anti-human acyl-Coenzyme A dehydrogenase, short/branched chain polyclonal Antibody
Product Synonym Names
acyl-Coenzyme A dehydrogenase; short/branched chain; ACADSB; 2-MEBCAD; ACAD7; SBCAD
Product Gene Name
anti-ACADSB antibody
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Species Reactivity
Human, Mouse, Rat
Purity/Purification
Antigen Affinity Purified
Storage Buffer
PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20 degree C, Avoid freeze / thaw cycles.
Santa Cruz Alternative
Potential replacement for Santa Cruz Biotechnology antibody catalog# sc-104796 / sc-104797
ISO Certification
Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.
Other Notes
Small volumes of anti-ACADSB 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.
Applications Tested/Suitable for anti-ACADSB antibody
ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)
NCBI/Uniprot data below describe general gene information for ACADSB. It may not necessarily be applicable to this product.
NCBI Accession #
AAH13756.1
[Other Products]
UniProt Secondary Accession #
Q5SQN6; Q96CX7; B4DQ51[Other Products]
UniProt Related Accession #
P45954[Other Products]
Molecular Weight
36,025 Da[Similar Products]
NCBI Official Full Name
Acyl-Coenzyme A dehydrogenase, short/branched chain
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; 2-methylbutyryl-coenzyme A dehydrogenase; 2-methyl branched chain acyl-CoA dehydrogenase; acyl-Coenzyme A dehydrogenase, short/branched chain
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
Protein Family
Short/branched chain specific acyl-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: Oxidoreductase; Mitochondrial; Amino Acid Metabolism - valine, leucine and isoleucine degradation; EC 1.3.8.5; Lipid Metabolism - fatty acid
Chromosomal Location of Human Ortholog: 10q26.13
Cellular Component: mitochondrion; mitochondrial matrix
Molecular Function: acyl-CoA dehydrogenase activity; FAD binding
Biological Process: branched chain family amino acid catabolic process; fatty acid metabolic process
Disease: 2-methylbutyryl-coa Dehydrogenase Deficiency
Research Articles on ACADSB
1. G mutation before knowing whether the optimal screening cut-off would minimize true positives or false negatives for SBCADD associated with this mutation.">the c.1165 A>G mutation before knowing whether the optimal screening cut-off would minimize true positives or false negatives for SBCADD associated with this mutation.
Precautions
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Disclaimer
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