Short chain 3-hydroxyacyl-CoA dehydrogenase, mitochondrial antibody can be used for the detection of Short chain 3-hydroxyacyl-CoA dehydrogenase, mitochondrial by Western Blot.
限制
仅限研究用
状态
Liquid
浓度
1 mg/mL
缓冲液
Phosphate-Buffered Saline. No preservatives added.
储存液
Without preservative
注意事项
As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.
储存条件
4 °C/-20 °C
储存方法
HADHSC antibody can be stored at 4 °C for short term (weeks). Long term storage should be at -20 °C.
抗原
HADH
(Hydroxyacyl-CoA Dehydrogenase (HADH))
别名
HADHSC
背景
FUNCTION: Plays an essential role in the mitochondrial beta-oxidation of short chain fatty acids. Exerts it highest activity toward 3-hydroxybutyryl-CoA.
DISEASE: Defects in HADHSC are the cause of 3-alpha-hydroxyacyl-CoA dehydrogenase deficiency (HAD deficiency) [MIM:609609]. HAD deficiency is a metabolic disorder with various clinical presentations including hypoglycemia, hepatoencephalopathy, myopathy or cardiomyopathy, and in some cases sudden death.