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GCS1 Rabbit anti-Human, Mouse, Rat, Polyclonal, Proteintech Non-distribution product as customer accommodation.

Rabbit Polyclonal Antibody

Manufacturer:  Proteintech Group IncSupplier Diversity Partner 178591AP

Catalog No. 50-173-1837


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Description

Description

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.
Specifications

Specifications

GCS1
0.13 mg/mL
PBS with 50% glycerol and 0.02% sodium azide; pH 7.3
O88941, Q13724, Q80UM7
GCS1, MOGS, Processing A glucosidase I
Rabbit
IgG
150 μL
-20°C
Primary
57377, 7841, 78947
Immunohistochemistry (Paraffin), Western Blot
Unconjugated
MOGS
Liquid
Mogs
GCS1 Fusion Protein Ag12321
Antigen Affinity Chromatography
RUO
Antibody
Polyclonal
Human, Mouse, Rat
Documents
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