Anti-ACADSB antibody (AA29867)

$287.00


Alternative names Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial, SBCAD, EC 1.3.8.5, 2-methyl branched chain acyl-CoA dehydrogenase, 2-MEBCAD, 2-methylbutyryl-coenzyme A dehydrogenase, 2-methylbutyryl-CoA dehydrogenase, ACADSB, ACDSB_HUMAN, P45954, 36
Source 432
Species Reactivity Human, Mouse, Rat
Applications IHC, WB
Host Rabbit
Class Polyclonal
Conjugated Unconjugated


Description

Product name Anti-ACADSB antibody (AA29867)
Catalog number AA29867
Brand family Ango
Product type Primary Antibody
Antibody Alternative Names Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial antibody, SBCAD antibody, EC 1.3.8.5 antibody, 2-methyl branched chain acyl-CoA dehydrogenase antibody, 2-MEBCAD antibody, 2-methylbutyryl-coenzyme A dehydrogenase antibody, 2-methylbutyryl-CoA dehydrogenase antibody, ACADSB antibody, ACDSB_HUMAN antibody


Biological Information

Immunogen Recombinant protein of human ACADSB
Concentration 1 mg/ml
Isotype IgG
Application Notes WB 1:500 - 1:2000 IHC 1:50 - 1:200
Purification method Purified by affinity chromatography


Target

Target Name ACADSB
SwissProt ID Link P45954
Function 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.
Subcellular Location Mitochondrion matrix.
Involvement in Disease Short/branched-chain acyl-CoA dehydrogenase deficiency: 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. The disease is caused by mutations affecting the gene represented in this entry.
Tissue Specificity Ubiquitous.
Subunit Structure Homotetramer.
Sequence Similarities Belongs to the acyl-CoA dehydrogenase family.


Poduct Presentation

Form Liquid
Preservative 0.02% (w/v) sodium azide
Storage buffer PBS with 50% glycerol, pH 7.3


Storage and Shipping Information

Storage Store at -20 or -80 C. Avoid multiple freeze/thaw cycles.