Invitrogen™ GCS1 Polyclonal Antibody

Rabbit Polyclonal Antibody

Supplier:  Invitrogen™ PA588726

Catalog No. PIPA588726

Description

Immunogen sequence: RWVLAWYRAR RAVTLHSAPP VLPADSSSPA VAPDLFWGTY RPHVYFGMKT RSPKPLLTGL MWAQQGTTPG TPKLRHTCEQ GDGVGPYGWE FHDGLSFGRQ HIQDGALRLT TEFVKRPGGQ HGGDWSWRVT VEPQDSGTSA LPLVSLFFYV VTDGKEVLLP EVGAKGQLKF ISGHTSELGD FRFTLLPPTS PGDTAPKYGS YNVFWTSNPG LPLLTEMVKS RLNSWFQHRP PGAPPERYLG LPGSLKWEDR GPSGQGQGQF L; Positive Samples: U-87MG, LO2, BxPC-3, OVCAR-3, Mouse Brain, Mouse Liver, Mouse Pancreas, Mouse Testis, Rat Liver; Cellular Location: Endoplasmic reticulum membrane, Single-pass type II membrane protein.

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: Affinity Chromatography
• Regulatory Status: RUO
• Gene ID (Entrez): 57377, 7841
• Content And Storage: -20°C, Avoid Freeze/Thaw Cycles
• Form: Liquid

• Applications: ELISA, Western Blot
• Concentration: 0.70 mg/mL
• Formulation: PBS with 50% glycerol and 0.01% thimerosal; pH 7.3
• Gene Accession No.: Q13724, Q80UM7
• Gene Symbols: MOGS
• Immunogen: Recombinant fusion protein containing a sequence corresponding to amino acids 60-320 of human MOGS (NP_0062932).
• Quantity: 100 μL
• Primary or Secondary: Primary
• Target Species: Human, Mouse, Rat
• Product Type: Antibody
• Isotype: IgG