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

Rabbit Polyclonal Antibody

Supplier:  Proteintech Group Inc 178591AP150UL

Catalog No. 50-173-1837

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
• Classification: Polyclonal
• Conjugate: Unconjugated
• Gene: MOGS
• Gene Alias: GCS1, MOGS, Processing A glucosidase I
• Host Species: Rabbit
• Purification Method: Antigen Affinity Chromatography
• Regulatory Status: RUO
• Gene ID (Entrez): 57377, 7841, 78947
• Content And Storage: -20°C
• Form: Liquid

• Applications: Immunohistochemistry (Paraffin), Western Blot
• Concentration: 0.13 mg/mL
• Formulation: PBS with 50% glycerol and 0.02% sodium azide; pH 7.3
• Gene Accession No.: O88941, Q13724, Q80UM7
• Gene Symbols: Mogs
• Immunogen: GCS1 Fusion Protein Ag12321
• Quantity: 150 μL
• Primary or Secondary: Primary
• Target Species: Human, Mouse, Rat
• Product Type: Antibody
• Isotype: IgG