Invitrogen™ GCS1 Polyclonal Antibody

Rabbit Polyclonal Antibody

Supplier:  Invitrogen™ PA582449

Catalog No. PIPA582449

Description

Immunogen sequence: LESHAEGFRE RFEKTFQLKE KGLSSGEQVL GQAALSGLLG GIGYFYGQGL VLPDIGVEGS EQKVDPALFP PVPLFTAVPS RSFFPRGFLW DEGFHQLVVQ RWDPSLTREA LGHWLGLLNA DGWIG.

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: 1810017N02Rik; AI181835; CDG2B; CWH41; DER7; GCS1; Glucosidase 1; glucosidase I; glycoprotein-processing glucosidase I; Mannosyl-oligosaccharide glucosidase; Mogs; Processing A-glucosidase I
• Host Species: Rabbit
• Purification Method: Antigen affinity chromatography
• Regulatory Status: RUO
• Gene ID (Entrez): 7841
• Content And Storage: Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
• Form: Liquid

• Applications: Immunohistochemistry (Paraffin), Western Blot
• Concentration: 0.1 mg/mL
• Formulation: PBS with 40% glycerol and 0.02% sodium azide; pH 7.2
• Gene Accession No.: Q13724
• Gene Symbols: MOGS
• Immunogen: Recombinant protein corresponding to Human MOGS.
• Quantity: 100 μL
• Primary or Secondary: Primary
• Target Species: Human
• Product Type: Antibody
• Isotype: IgG

Safety and Handling

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