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Invitrogen
GCS1 Polyclonal Antibody
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产品信息
PA5-12128
产品规格
宿主/亚型
Rabbit
/ IgG
分类
Polyclonal
类型
Antibody
抗原
KLH conjugated synthetic peptide between 1-30 amino acids from the N-terminal region of human GCS1
偶联物
形式
Liquid
浓度
Lot-specific
保存条件
-20°C, Avoid Freeze/Thaw Cycles
运输条件
Wet ice
RRID
靶标信息
GCS1 cleaves the distal alpha 1,2-linked glucose residue from the Glc(3)Man(9)GlcNAc(2) oligosaccharide precursor in a highly specific manner. Defects in GCS1 are the cause of type IIb congenital disorder of glycosylation (CDGIIb). This syndrome is also known as glucosidase I deficiency and is characterized by marked generalized hypotonia and hypomotility of the neonate, dysmorphic features, including a prominent occiput, short palpebral fissures, retrognathia, high arched palate, generalized edema, and hypoplastic genitalia. Symptoms include hepatomegaly, hypoventilation, feeding problems and seizures. The clinical course is progressive and survival is at most a few months.
仅用于科研。不用于诊断过程。未经明确授权不得转售。
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