HADHA Mouse Monoclonal Antibody (Biotin conjugated) [Clone ID: OTI7B3]

Specifications

Product Data
Clone Name	OTI7B3
Applications	WB
Recommend Dilution	WB 1:500
Reactivity	Human, Rat, Mouse
Host	Mouse
Clonality	Monoclonal
Immunogen	Human recombinant protein fragment corresponding to amino acids 106-325 of human HADHA (NP_000173) produced in E.coli.
Formulation	PBS (pH 7.3) containing 1% BSA, 50% glycerol and 0.02% sodium azide.
Concentration	0.5 mg/ml
Purification	Purified from mouse ascites fluids or tissue culture supernatant by affinity chromatography (protein A/G)
Conjugation	Biotin
Storage Condition	Store at -20°C as received.
Predicted Protein Size	83 kDa
Gene Name	hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit
Database Link	NP_000173 Entrez Gene 97212 MouseEntrez Gene 170670 RatEntrez Gene 3030 Human UniProt ID P40939
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]
Synonyms	ECHA; GBP; HADH; LCEH; LCHAD; MTPA; TP-ALPHA
Reference Data
Protein Families	Druggable Genome
Protein Pathways	beta-Alanine metabolism, Biosynthesis of unsaturated fatty acids, Butanoate metabolism, Fatty acid elongation in mitochondria, Fatty acid metabolism, Limonene and pinene degradation, Lysine degradation, Metabolic pathways, Propanoate metabolism, Tryptophan metabolism, Valine, leucine and isoleucine degradation

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