Total Glucosidase IIβ Cell-Based Colorimetric ELISA Kit
-KA3542C
主要信息
Reactivity
Human, Mouse
Applications
ELISA
Conjugate/Modification
Unmodified
详细信息
储存
2-8°C/6 months
修饰
Unmodified
检测方法
Colorimetric
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抗原&靶点信息
基因名称:
PRKCSH
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别名:
Glucosidase 2 subunit beta ;
80K-H protein ;
Glucosidase II subunit beta ;
Protein kinase C substrate 60.1 kDa protein heavy chain ;
PKCSH ;
80K-H protein ;
Glucosidase II subunit beta ;
Protein kinase C substrate 60.1 kDa protein heavy chain ;
PKCSH ;
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背景:
disease:Defects in PRKCSH are a cause of polycystic liver disease (PCLD) [MIM:174050]. PCLD is an autosomal dominant disorder and is characterized by the presence of multiple liver cysts of biliary epithelial origin. PCLD is a distinct clinical and genetic entity that can occur independently from autosomal dominant polycystic kidney disease (ADPKD) [MIM:173900], which in a considerable but uncertain proportion of cases is associated with hepatic cysts.,function:Regulatory subunit of glucosidase II.,pathway:Glycan metabolism; N-glycan metabolism.,similarity:Contains 1 PRKCSH domain.,similarity:Contains 2 EF-hand domains.,subunit:Heterodimer of a catalytic alpha subunit (GANAB) and a beta subunit (PRKCSH). Binds glycosylated PTPRC.,
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功能:
intracellular signaling cascade, protein kinase cascade,
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细胞定位:
Endoplasmic reticulum .
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