Anti-ACADL antibody (AA24014)

$287.00


Alternative names Long-chain specific acyl-CoA dehydrogenase, mitochondrial, LCAD, EC 1.3.8.8, ACADL, ACADL_HUMAN, P28330, 33
Source 430
Species Reactivity Human, Mouse
Applications IHC, WB
Host Rabbit
Class Polyclonal
Conjugated Unconjugated


Description

Product name Anti-ACADL antibody (AA24014)
Catalog number AA24014
Brand family Ango
Product type Primary Antibody
Antibody Alternative Names Long-chain specific acyl-CoA dehydrogenase, mitochondrial antibody, LCAD antibody, EC 1.3.8.8 antibody, ACADL antibody, ACADL_HUMAN antibody


Biological Information

Immunogen Recombinant protein of human ACADL
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 ACADL
SwissProt ID Link P28330
Subcellular Location Mitochondrion matrix.
Involvement in Disease Acyl-CoA dehydrogenase very long-chain deficiency: An inborn error of mitochondrial fatty acid beta-oxidation which leads to impaired long-chain fatty acid beta-oxidation. It is clinically heterogeneous, with three major phenotypes: a severe childhood form characterized by early onset, high mortality and high incidence of cardiomyopathy a milder childhood form with later onset, characterized by hypoketotic hypoglycemia, low mortality and rare cardiomyopathy an adult form, with isolated skeletal muscle involvement, rhabdomyolysis and myoglobinuria, usually triggered by exercise or fasting. The disease is caused by mutations affecting the gene represented in this entry.
Post-Translational Modification Acetylation at Lys-318 and Lys-322 in proximity of the cofactor-binding sites strongly reduces catalytic activity. These sites are deacetylated by SIRT3.
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.