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Home Antibody All anti-HADHA antibodies

Anti-HADHA Antibody

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Specifications Citations Related Products Product Documents
SKU Description Amount Price Availability*  
TA318956
  • Rabbit polyclonal anti-TFP1/HADHA antibody
  • Free Sample of Positive Control: HEK293T cell transient overexpression lysate (LC400065) , 20ug Explanation
100ug 325 3-7 Days
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WB(1)

OriGene Data

ImmunogenSynthetic peptide surrounding amino acid 750 of human TFP1
Clone Name IsotypeRabbit IgG
Species ReactivityHuman, mouse, rat Concentration0.5 mg/ml
Guaranteed Application *WB Suggested DilutionsWB: 0.5-4 ug/ml
Buffer100 µg (0.5 mg/ml) affinity purified rabbit polyclonal antibody in 1X phosphate-buffered saline (PBS) containing 30% glycerol, 0.5% BSA, and 0.01% thimerosal.
Purification Affinity purified

Reference Data

Target NameHomo sapiens hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit (HADHA)
Alternative NameECHA; GBP; HADH; LCEH; LCHAD; MTPA; TP-ALPHA
Database LinkNP_000173
Entrez Gene 3030 Human
Entrez Gene 97212 Mouse
Entrez Gene 170670 Rat
FunctionMitochondrial Trifunctional Protein (TFP) is a multienzyme complex of the beta-oxidation cycle. TFP deficiency is a clinically heterogeneous disorder with phenotypes of different severity. The spectrum of diseases range from severe neonatal/infantile cardiomyopathy and early death to mild chronic progressive sensorimotor poly-neuropathy with episodic rhabdomyolysis. Human TFP is an octomer composed of four alpha-subunits and four beta-subunits. Mutations in either subunits may result in general TFP deficiency with reduced activity of all enzymes.
Related PathwayDruggable Genome Fatty acid elongation in mitochondriaFatty acid metabolismValine, leucine and isoleucine degradationLysine degradationTryptophan metabolismbeta-Alanine metabolismMore Pathways >>

* Availability is in business days
* OriGene provides validated application data and protocol, with money back guarantee.

WB Image
Western blot analysis of TFP-1 expression with lysate from Jurkat cells (Lane 1,2), 3T3 cells (Lane 3) and rat kidney (Lane 4).

 

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