HADHA
Reaktivität: Human
WB, IP
Wirt: Kaninchen
Polyclonal
unconjugated
Applikationshinweise
ELISA: 1/1,000Western blotting: 1/100approx. 1/500. Other applications not tested. Optimal dilutions are dependent on conditions and should be determined by the user.
Beschränkungen
Nur für Forschungszwecke einsetzbar
Format
Liquid
Konzentration
0.25 mg/mL
Buffer
PBS with 0.09 % (W/V) Sodium Azide as preservative
Konservierungsmittel
Sodium azide
Vorsichtsmaßnahmen
This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
Handhabung
Avoid repeated freezing and thawing.
Lagerung
4 °C/-20 °C
Informationen zur Lagerung
Store undiluted at 2-8 °C for one month or (in aliquots) at -20 °C for longer.
Target
HADHA
(Hydroxyacyl-Coenzyme A Dehydrogenase/3-Ketoacyl-Coenzyme A Thiolase/enoyl-Coenzyme A Hydratase (Trifunctional Protein), alpha Subunit (HADHA))
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. HADHA is 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.Synonyms: HADH, Long chain 3-hydroxyacyl-CoA dehydrogenase, Long-chain enoyl-CoA hydratase, TP-alpha, Trifunctional enzyme subunit alpha mitochondrial