Name: U-Blot® ECHA Rabbit mAb
SKU: WR6034
Price: 268 USD
Availability: InStock

Product details

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. [provided by RefSeq , Jul 2008] ,

Specifications

Size	50μL/100μL
Target	ECHA
Reactivity	Human, Mouse, Rat
Applications	WB, IHC, IF, IP, ELISA
MW(Calculated)	74kD
MW(Observed)	74kD
Host Species	Rabbit
Isotype	IgG,Kappa
Conjugate/Modification	Unmodified
Recommended Dilution Ratio	IHC 1:200-1:1000; WB 1:2000-1:10000; IF 1:200-1:1000; ELISA 1:5000-1:20000; IP 1:50-1:200; / Note: For IHC, we suggest antigen retrieval with TE buffer pH 9.0 (Cat#WT1004)
Formulation	PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA
Purification	Recombinant Antibody expressed in animal component-free (ACF) media, purified via Protein A affinity chromatography.
Storage	-15°C to -25°C/1 year(Do not lower than -25°C)
Concentration
Clonality	Monoclonal
Clone Number	RM1955R
Immunogen	The specific immunogen used to produce this antibody is proprietary information.
Specificity	Endogenous
Gene Name	HADHA HADH
Protein Name	ECHA
Other Name	Trifunctional enzyme subunit alpha, mitochondrial; / 78 kDa gastrin-binding protein; / TP-alpha; / [Includes: Long-chain enoyl-CoA hydratase; / Long chain 3-hydroxyacyl-CoA dehydrogenase; / ]
Species	Human
Gene ID	3030
Uniprot	P40939,
Species.1	Mouse
Gene ID.1	97212
Uniprot.1	Q8BMS1,
Species.2	Rat
Gene ID.2	170670
Uniprot.2	Q64428
Cellular Localization	Mitochondrion . Mitochondrion inner membrane . Protein stability and association with mitochondrion inner membrane do not require HADHB. .
Tissue Expression	Epithelium,Lymph,
Signaling_pathway
Research Areas	>>Fatty acid elongation; / >>Fatty acid degradation; / >>Valine, leucine and isoleucine degradation; / >>Lysine degradation; / >>Tryptophan metabolism; / >>beta-Alanine metabolism; / >>Propanoate metabolism; / >>Butanoate metabolism; / >>Metabolic pathways; / >>Fatty acid metabolism
Function	Catalytic activity:(3S)-3-hydroxyacyl-CoA = trans-2(or 3)-enoyl-CoA + H(2)O.,Catalytic activity:(S)-3-hydroxyacyl-CoA + NAD(+) = 3-oxoacyl-CoA + NADH.,Disease:Defects in HADHA are a cause of maternal acute fatty liver of pregnancy (AFLP) [MIM:609016]. AFLP is a severe maternal illness occurring during pregnancies with affected fetuses. This disease is associated with LCHAD deficiency and characterized by sudden unexplained infant death or hypoglycemia and abnormal liver enzymes (Reye-like syndrome).,Disease:Defects in HADHA are a cause of trifunctional protein deficiency (TFP deficiency) [MIM:609015]. The clinical manifestations are very variable and include hypoglycemia, cardiomyopathy and sudden death. Phenotypes with mainly hepatic and neuromyopathic involvement can also be distinguished. Biochemically, TFP deficiency is defined by the loss of all enzyme activities of the TFP complex.,Disease:Defects in HADHA are the cause of long-chain 3-hydroxyl-CoA dehydrogenase deficiency (LCHAD deficiency) [MIM:609016]. The clinical features are very similar to TFP deficiency. Biochemically, LCHAD deficiency is characterized by reduced long-chain 3-hydroxyl-CoA dehydrogenase activity, while the other enzyme activities of the TFP complex are normal or only slightly reduced.,Function:Bifunctional subunit.,pathway:Lipid metabolism; fatty acid beta-oxidation.,similarity:Belongs to the enoyl-CoA hydratase/isomerase family.,similarity:In the central section; belongs to the 3-hydroxyacyl-CoA dehydrogenase family.,similarity:In the N-terminal section; belongs to the enoyl-CoA hydratase/isomerase family.,subunit:Octamer of 4 alpha (HADHA) and 4 beta (HADHB) subunits.,