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HADHA (Trifunctional Enzyme Subunit alpha, Mitochondrial, 78kD Gastrin-binding Protein, TP-alpha, HADH, Long-chain Enoyl-CoA Hydratase, Long Chain 3-hydroxyacyl-CoA Dehydrogenase)

HADHA (Trifunctional Enzyme Subunit alpha, Mitochondrial, 78kD Gastrin-binding Protein, TP-alpha, HADH, Long-chain Enoyl-CoA Hydratase, Long Chain 3-hydroxyacyl-CoA Dehydrogenase)

Cat no: H1500-11A

Supplier: United States Biological
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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. Applications: Suitable for use in Western Blot. Other applications not tested. Recommended Dilution: Western Blot: 0.5-4ug/ml Optimal dilutions to be determined by the researcher. Storage and Stability: May be stored at 4 degrees C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degrees C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Catalogue number: H1500-11A
Reactivities: Human, Mouse, Rat
Hosts: Rabbit
Applications: Western Blot
Size: 100ug
Form: Supplied as a liquid in PBS, 0.5% BSA, 0.01% thimerosal, 30% glycerol.
P type: Pab
Isotype: IgG
Purity: Purified by affinity chromatography.
Additional info: Recognizes human TFP1 at ~84kD. Species Crossreactivity: mouse and rat.

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