This webpage contains legacy information. The product is either no longer available from the supplier or has been delisted at Labome.
product summary
company name :
Invitrogen
other brands :
NeoMarkers, Lab Vision, Endogen, Pierce, BioSource International, Zymed Laboratories, Caltag, Molecular Probes, Research Genetics, Life Technologies, Applied Biosystems, GIBCO BRL, ABgene, Dynal, Affinity BioReagents, Nunc, Invitrogen, NatuTec, Oxoid, Richard-Allan Scientific, Arcturus, Perseptive Biosystems, Proxeon, eBioscience
product type :
antibody
product name :
GCS1 Polyclonal Antibody
catalog :
PA5-88726
quantity :
100 uL
price :
US 464.00
clonality :
polyclonal
host :
domestic rabbit
conjugate :
nonconjugated
reactivity :
human, mouse, rat
application :
western blot, ELISA
product information
Product Type :
Antibody
Product Name :
GCS1 Polyclonal Antibody
Catalog # :
PA5-88726
Quantity :
100 uL
Price :
US 464.00
Clonality :
Polyclonal
Purity :
Affinity Chromatography
Host :
Rabbit
Reactivity :
Human, Mouse, Rat
Applications :
ELISA: 1 ug/mL, Western Blot: 1:500-1:2,000
Species :
Human, Mouse, Rat
Isotype :
IgG
Storage :
-20 C, Avoid Freeze/Thaw Cycles
Description :
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.
Immunogen :
Recombinant fusion protein containing a sequence corresponding to amino acids 60-320 of human MOGS (NP_0062932)
Format :
Liquid
Applications w/Dilutions :
ELISA: 1 ug/mL, Western Blot: 1:500-1:2,000
Aliases :
1810017N02Rik; AI181835; CDG2B; CWH41; DER7; GCS1; Glucosidase 1; glucosidase I; glycoprotein-processing glucosidase I; Mannosyl-oligosaccharide glucosidase; Mogs; Processing A-glucosidase I
company information
Invitrogen
Thermo Fisher Scientific
81 Wyman Street
Waltham, MA USA 02451
https://www.thermofisher.com
800-678-5599
headquarters: USA