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

Rabbit Polyclonal Antibody

Catalog Number	Mfr. No.	Quantity
50-173-1837	Proteintech Group Inc 178591AP150UL	150 μL

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

• Antigen: GCS1
• Applications: Immunohistochemistry (Paraffin), Western Blot
• Conjugate: Unconjugated
• Host Species: Rabbit
• Target Species: Human, Mouse, Rat
• Gene Accession No.: O88941, Q13724, Q80UM7
• Gene ID (Entrez): 57377, 7841, 78947
• Immunogen: GCS1 Fusion Protein Ag12321
• Primary or Secondary: Primary
• Content And Storage: -20°C
• Gene: MOGS

• Concentration: 0.13 mg/mL
• Classification: Polyclonal
• Form: Liquid
• Regulatory Status: RUO
• Formulation: PBS with 50% glycerol and 0.02% sodium azide; pH 7.3
• Gene Alias: GCS1, MOGS, Processing A glucosidase I
• Gene Symbols: Mogs
• Isotype: IgG
• Purification Method: Antigen Affinity Chromatography
• Product Type: Antibody