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Invitrogen™ GCS1 Polyclonal Antibody

Rabbit Polyclonal Antibody

Supplier:  Invitrogen™ PA564937

Catalog No. PIPA564937


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Description

Description

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

Specifications

GCS1
Polyclonal
Unconjugated
MOGS
1810017N02Rik; AI181835; CDG2B; CWH41; DER7; GCS1; Glucosidase 1; glucosidase I; glycoprotein-processing glucosidase I; Mannosyl-oligosaccharide glucosidase; Mogs; Processing A-glucosidase I
Rabbit
Antigen affinity chromatography
RUO
7841
Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
Liquid
Western Blot, Immunocytochemistry
0.05 mg/mL
PBS with 40% glycerol and 0.02% sodium azide; pH 7.2
Q13724
MOGS
Recombinant Human GCS1. Recombinant protein control fragment (Product #RP-90142).
100 μL
Primary
Human
Antibody
IgG
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