Thermo Fisher Scientific GCS1 Polyclonal Antibody

Applications

Tested Dilution

Publications

Western Blot (WB)

1:500-1:3,000

-

Product Specifications

Species Reactivity

Human

Host/Isotype

Rabbit / IgG

Class

Polyclonal

Type

Antibody

Immunogen

Recombinant fragment corresponding to a region within amino acids 136 and 355 of Human Glucosidase I if (typeof window.$mangular === undefined || !window.$mangular) { window.$mangular = {}; } $mangular.antigenJson = \[{targetFamily:GCS1,uniProtId:Q13724-1,ncbiNodeId:9606,antigenRange:136-355,antigenLength:837,antigenImageFileName:PA5-28312_GCS1_Q13724-1_Rabbit.svg,antigenImageFileNamePDP:PA5-28312_GCS1_Q13724-1_Rabbit_PDP.jpeg,sortOrder:1}\]; $mangular.isB2BCMGT = false; $mangular.isEpitopesModalImageMultiSizeEnabled = true;

View immunogen .st0{fill:#FFFFFF;} .st1{fill:#1E8AE7;}

Conjugate

Unconjugated Unconjugated Unconjugated

Form

Liquid

Concentration

1 mg/mL

Purification

Antigen affinity chromatography

Storage buffer

0.1M tris glycine, pH 7, with 10% glycerol

Contains

0.01% thimerosal

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_2545788

Product Specific Information

Recommended positive controls: 293T, A431, H1299, HeLaS3, HepG2, Molt-4, Raji.

Predicted reactivity: Mouse (87%).

Store product as a concentrated solution. Centrifuge briefly prior to opening the vial.

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.

⚠WARNING: This product can expose you to chemicals including mercury, which is known to the State of California to cause birth defects or other reproductive harm. For more information go to www.P65Warnings.ca.gov.

For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.