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GCS1 Rabbit anti-Human, Mouse, Rat, Polyclonal, Proteintech
SDP

Catalog No. 501731837 Shop All Proteintech Group Inc Products
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50-173-1837 150 μL
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Catalog No. 50-173-1837 Supplier Proteintech Group Inc Supplier No. 178591AP150UL
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Description

Rabbit Polyclonal Antibody

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

Antigen GCS1
Applications Immunohistochemistry (Paraffin), Western Blot
Classification Polyclonal
Concentration 0.13 mg/mL
Conjugate Unconjugated
Formulation PBS with 50% glycerol and 0.02% sodium azide; pH 7.3
Gene MOGS
Gene Accession No. O88941, Q13724, Q80UM7
Gene Alias GCS1, MOGS, Processing A glucosidase I
Gene Symbols Mogs
Host Species Rabbit
Immunogen GCS1 Fusion Protein Ag12321
Purification Method Antigen Affinity Chromatography
Quantity 150 μL
Regulatory Status RUO
Primary or Secondary Primary
Gene ID (Entrez) 57377, 7841, 78947
Target Species Human, Mouse, Rat
Content And Storage -20°C
Product Type Antibody
Form Liquid
Isotype IgG
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