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Invitrogen™ GCS1 Polyclonal Antibody
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Catalog No. PIPA564937
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PIPA564937 100 μL
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Catalog No. PIPA564937 Supplier Invitrogen™ Supplier No. PA564937
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Description

Rabbit Polyclonal Antibody

Immunogen sequence: LESHAEGFRER FEKTFQLKEK GLSSGEQVLG QAALSGLLGG IGYFYGQGLV LPDIGVEGSE QKVDPALFPP VPLFTAVPSR SFFPRGFLWD EGFHQLVVQR WDPSLTREAL GHWLGLLNAD GWIG Highest antigen sequence identity to the following orthologs - mouse 89%, rat 88%.

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.
TRUSTED_SUSTAINABILITY

Specifications

Antigen GCS1
Applications Western Blot, Immunocytochemistry
Classification Polyclonal
Concentration 0.05 mg/mL
Conjugate Unconjugated
Formulation PBS with 40% glycerol and 0.02% sodium azide; pH 7.2
Gene MOGS
Gene Accession No. Q13724
Gene Alias 1810017N02Rik; AI181835; CDG2B; CWH41; DER7; GCS1; Glucosidase 1; glucosidase I; glycoprotein-processing glucosidase I; Mannosyl-oligosaccharide glucosidase; Mogs; Processing A-glucosidase I
Gene Symbols MOGS
Host Species Rabbit
Immunogen Recombinant Human GCS1. Recombinant protein control fragment (Product #RP-90142).
Purification Method Antigen affinity chromatography
Quantity 100 μL
Regulatory Status RUO
Primary or Secondary Primary
Gene ID (Entrez) 7841
Target Species Human
Content And Storage Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
Product Type Antibody
Form Liquid
Isotype IgG
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Safety and Handling

WARNING: Cancer - www.P65Warnings.ca.gov
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