Thermo Fisher Scientific GCS1 Polyclonal Antibody

Applications

Tested Dilution

Publications

Western Blot (WB)

0.04-0.4 µg/mL

-

Immunocytochemistry (ICC/IF)

0.25-2 µg/mL

-

Product Specifications

Species Reactivity

Human

Host/Isotype

Rabbit / IgG

Class

Polyclonal

Type

Antibody

Immunogen

Recombinant Human GCS1. Recombinant protein control fragment (Product #RP-90142). if (typeof window.$mangular === undefined || !window.$mangular) { window.$mangular = {}; } $mangular.antigenJson = \[{targetFamily:GCS1,uniProtId:Q13724-1,ncbiNodeId:9606,antigenRange:361-485,antigenLength:837,antigenImageFileName:PA5-64937_GCS1_Q13724-1_Rabbit.svg,antigenImageFileNamePDP:PA5-64937_GCS1_Q13724-1_Rabbit_PDP.jpeg,sortOrder:1}\]; $mangular.isB2BCMGT = false; $mangular.isEpitopesModalImageMultiSizeEnabled = true;

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Conjugate

Unconjugated Unconjugated Unconjugated

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.