Peptide ELISA: Detection Limit: 1/8000. Western blot: 1-3 μg/mL. Detects a band of Approx 70 kDa in Human and Mouse Liver lysates. An additional band of unknown identity was also consistently observed at 20 kDa. Thisband was successfully blocked by incubation with the immunizing peptide. Other applications not tested. Optimal dilutions are dependent on conditions and should be determined by the user.
限制
仅限研究用
浓度
0.5 mg/mL
缓冲液
Tris saline, pH 7.3 containing 0.02 % Sodium Azide as preservative and 0.5 % BSA as stabilizer
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 the antibody undiluted at 2-8 °C for one month or (in aliquots) at -20 °C for longer.
Carnitine palmitoyltransferase II (CPT2) together with carnitine palmitoyltransferase I oxidizes long-chain fatty acids in the mitochondria. Defects in CPT2 are the cause of carnitine palmitoyltransferase 2 deficiency (CPT2D) also known as CPT-II deficiency or CPT2 deficiency. CPT2D is an autosomal recessive disorder characterized by recurrent myoglobinuria, episodes of muscle pain, stiffness, and rhabdomyolysis. These symptoms are triggered by prolonged exercise, fasting or viral infection and patients are usually young adults. In addition to this classical, late-onset, muscular type, a hepatic or hepatocardiomuscular form has been reported in infants. Clinical pictures in these children or neonates include hypoketotic hypoglycemia, liver dysfunction, cardiomyopathy and sudden death.Synonyms: CPT II, Carnitine O-palmitoyltransferase 2, Carnitine palmitoyltransferase II, mitochondrial