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.
限制
仅限研究用
状态
Liquid
浓度
0.25 mg/mL
缓冲液
PBS with 0.09 % (W/V) Sodium Azide as preservative
储存液
Sodium azide
注意事项
This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
注意事项
Avoid repeated freezing and thawing.
储存条件
4 °C/-20 °C
储存方法
Store undiluted at 2-8 °C for one month or (in aliquots) at -20 °C for longer.
抗原
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