Thermo Fisher Scientific GCS1 Polyclonal Antibody

Applications

Tested Dilution

Publications

Western Blot (WB)

1:500-1:2,000

-

ELISA (ELISA)

1 µg/mL

-

Product Specifications

Species Reactivity

Human, Mouse, Rat

Host/Isotype

Rabbit / IgG

Class

Polyclonal

Type

Antibody

Immunogen

Recombinant fusion protein containing a sequence corresponding to amino acids 60-320 of human MOGS (NP_0062932) if (typeof window.$mangular === undefined || !window.$mangular) { window.$mangular = {}; } $mangular.antigenJson = \[{targetFamily:GCS1,uniProtId:Q13724-1,ncbiNodeId:9606,antigenRange:60-320,antigenLength:837,antigenImageFileName:PA5-88726_GCS1_Q13724-1_Rabbit.svg,antigenImageFileNamePDP:PA5-88726_GCS1_Q13724-1_Rabbit_PDP.jpeg,sortOrder:1}\]; $mangular.isB2BCMGT = false; $mangular.isEpitopesModalImageMultiSizeEnabled = true;

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

Conjugate

Unconjugated Unconjugated Unconjugated

Form

Liquid

Concentration

0.70 mg/mL

Purification

Affinity Chromatography

Storage buffer

PBS, pH 7.3, with 50% glycerol

Contains

0.01% thimerosal

Storage conditions

-20° C, Avoid Freeze/Thaw Cycles

Shipping conditions

Wet ice

RRID

AB_2805092

Product Specific Information

Immunogen sequence: RWVLAWYRAR RAVTLHSAPP VLPADSSSPA VAPDLFWGTY RPHVYFGMKT RSPKPLLTGL MWAQQGTTPG TPKLRHTCEQ GDGVGPYGWE FHDGLSFGRQ HIQDGALRLT TEFVKRPGGQ HGGDWSWRVT VEPQDSGTSA LPLVSLFFYV VTDGKEVLLP EVGAKGQLKF ISGHTSELGD FRFTLLPPTS PGDTAPKYGS YNVFWTSNPG LPLLTEMVKS RLNSWFQHRP PGAPPERYLG LPGSLKWEDR GPSGQGQGQF L; Positive Samples: U-87MG, LO2, BxPC-3, OVCAR-3, Mouse Brain, Mouse Liver, Mouse Pancreas, Mouse Testis, Rat Liver; Cellular Location: Endoplasmic reticulum membrane, Single-pass type II membrane protein

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.