Home Antibody All anti-HADHA antibodies
Also for HADHA (NM_000182)
|Synthetic peptide surrounding amino acid 750 of human TFP1|
|Human, mouse, rat
||WB: 0.5-4 ug/ml
|100 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.|
|Homo sapiens hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit (HADHA)|
|ECHA; GBP; HADH; LCEH; LCHAD; MTPA; TP-ALPHA|
|Mitochondrial 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.|
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Western blot analysis of TFP-1 expression with lysate from Jurkat cells (Lane 1,2), 3T3 cells (Lane 3) and rat kidney (Lane 4).