HADHA antibody
Product Specifications
Background
This gene encodes the alpha subunit of the mitochondrial trifunctional protein, which catalyzes the last three steps of mitochondrial beta-oxidation of long chain fatty acids. The mitochondrial membrane-bound heterocomplex is composed of four alpha and four beta subunits, with the alpha subunit catalyzing the 3-hydroxyacyl-CoA dehydrogenase and enoyl-CoA hydratase activities. Mutations in this gene result in trifunctional protein deficiency or LCHAD deficiency. The genes of the alpha and beta subunits of the mitochondrial trifunctional protein are located adjacent to each other in the human genome in a head-to-head orientation.
Synonyms
Trifunctional enzyme subunit alpha, mitochondrial|78 kDa gastrin-binding protein|Monolysocardiolipin acyltransferase|TP-alpha|HADHA|HADH
Gene ID
3030
UniProt
P40939
Host
Rabbit
Reactivity
Human, Mouse, Rat
Immunogen
Hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), alpha subunit
Target
HADHA
Clonality
Polyclonal
Isotype
IgG
Applications
ELISA, WB, IHC, IF
Field of Research
Cardiovascular, Metabolism
Purification
Immunogen affinity purified
Dilution
WB: 1:500 - 1:2000; IHC: 1:50 - 1:200; IF: 1:50 - 1:200
Purity
≥95% as determined by SDS-PAGE
Form
Liquid
Molecular Weight
88 kDa
Shipping Conditions
4°C with ice bag
Storage Conditions
PBS with 0.02% sodium azide and 50% glycerol pH 7.3, -20°C for 12 months (Avoid repeated freeze / thaw cycles.)
Tested Applications
ELISA, WB, IHC, IF
Immunogen Sequence
488-763aa
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