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Invitrogen™ GCS1 Polyclonal Antibody
Rabbit Polyclonal Antibody
Supplier: Invitrogen™ PA582449
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
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 |
Immunohistochemistry (Paraffin), Western Blot | |
0.1 mg/mL | |
PBS with 40% glycerol and 0.02% sodium azide; pH 7.2 | |
Q13724 | |
MOGS | |
Recombinant protein corresponding to Human MOGS. | |
100 μL | |
Primary | |
Human | |
Antibody | |
IgG |
Safety and Handling
WARNING: Cancer - www.P65Warnings.ca.gov
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