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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 positive control: HEK293T cell transient overexpression lysate (LC400065) , 20ug
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
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 Pathway

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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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