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Product Details
PA5-64937
Applications
Tested Dilution
Publications
Product Specifications
Species Reactivity
Human
Host/Isotype
Rabbit
/ IgG
Class
Polyclonal
Type
Antibody
Immunogen
Recombinant Human GCS1. Recombinant protein control fragment (Product #RP-90142).
Conjugate
Form
Liquid
Concentration
0.05 mg/mL
Purification
Antigen affinity chromatography
Storage buffer
PBS, pH 7.2, with 40% glycerol
Contains
0.02% sodium azide
Storage conditions
Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
Shipping conditions
Wet ice
RRID
AB_2663261
Product Specific Information
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%.
Target Information
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.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
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Bioinformatics
Protein Aliases: glucosidase I; Mannosyl-oligosaccharide glucosidase; Processing A-glucosidase I
Gene Aliases: CDG2B; CWH41; DER7; GCS1; MOGS
UniProt ID: (Human) Q13724
Entrez Gene ID: (Human) 7841
Performance Guarantee
If an Invitrogen™ antibody doesn't perform as described on our website or datasheet,we'll replace the product at no cost to you, or provide you with a credit for a future purchase.*
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