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折扣/货期| 货号：XW-8005 | 规格：0.05 mg | 目录价：￥11196.9 |
产品详情
* 以下信息仅供参考，详情请以原厂网站为准
产品名称:
HADHSC Antibody
别名:
HAD, HADHSC, SCHAD, Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial, Medium and short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase, HCDH, Short chain 3hydroxyacylCoA dehydrogenase, Medium and short chain L-3-hydroxyacyl-coenzyme A dehydrogenase
反应种属:
Human,Mouse,Rat
宿主来源:
Chicken
实验应用:
WB
克隆性:
Polyclonal
纯化方式:
Immunoaffinity Purified
偶联:
Unconjugated
产品浓度:
1 mg/mL
保存温度:
HADHSC antibody can be stored at 4˚C for short term (weeks). Long term storage should be at -20˚C. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.
运输温度:
Blue Ice
产品形式:
Liquid
存储溶液:
Phosphate-Buffered Saline. No preservatives added.
产地:
美国
生产商:
Prosci
功能与背景:
FUNCTION: Plays an essential role in the mitochondrial β-oxidation of short chain fatty acids. Exerts it highest activity toward 3-hydroxybutyryl-CoA.
DISEASE: Defects in HADHSC are the cause of 3-α-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.
DISEASE: Defects in HADHSC are the cause of 3-α-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.
Accession #:
NP_005318.1
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