Promotional price valid on web orders only. Your contract pricing may differ. Interested in signing up for a dedicated account number?
Learn More
  1. Home
  2. Products
  3. Antibodies
  4. Primary Antibodies
  5. All Primary Antibodies
  6. PIPA582449

Invitrogen™ GCS1 Polyclonal Antibody
GREENER_CHOICE

Catalog No. PIPA582449
Change view
Click to view available options
Quantity:
100 μL
1 product options available for selection
Product selection table with 1 available options. Use arrow keys to navigate and Enter or Space to select.
Catalog No. Quantity
PIPA582449 100 μL
Use arrow keys to navigate between rows. Press Enter or Space to select a product option. 1 options available.
1 options
Catalog No. PIPA582449 Supplier Invitrogen™ Supplier No. PA582449
Add to Cart
Add to Cart

Description

Rabbit Polyclonal Antibody

Immunogen sequence: LESHAEGFRE RFEKTFQLKE KGLSSGEQVL GQAALSGLLG GIGYFYGQGL VLPDIGVEGS EQKVDPALFP PVPLFTAVPS RSFFPRGFLW DEGFHQLVVQ RWDPSLTREA LGHWLGLLNA DGWIG Highest antigen sequence indentity to the following orthologs: Mouse - 89%, Rat - 88%.

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.
TRUSTED_SUSTAINABILITY

Specifications

Antigen GCS1
Applications Immunohistochemistry (Paraffin), Western Blot
Classification Polyclonal
Concentration 0.05 mg/mL
Conjugate Unconjugated
Formulation PBS with 40% glycerol and 0.02% sodium azide; pH 7.2
Gene MOGS
Gene Accession No. Q13724
Gene Alias 1810017N02Rik; AI181835; CDG2B; CWH41; DER7; GCS1; Glucosidase 1; glucosidase I; glycoprotein-processing glucosidase I; Mannosyl-oligosaccharide glucosidase; Mogs; Processing A-glucosidase I
Gene Symbols MOGS
Host Species Rabbit
Immunogen Recombinant protein fragment of Human MOGS (Product #RP-90142)
Purification Method Antigen affinity chromatography
Quantity 100 μL
Regulatory Status RUO
Primary or Secondary Primary
Gene ID (Entrez) 7841
Target Species Human
Content And Storage Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
Product Type Antibody
Form Liquid
Isotype IgG
Show More Show Less

Safety and Handling

WARNING: Cancer - www.P65Warnings.ca.gov
Product Title
Select an issue

By clicking Submit, you acknowledge that you may be contacted by Fisher Scientific in regards to the feedback you have provided in this form. We will not share your information for any other purposes. All contact information provided shall also be maintained in accordance with our Privacy Policy.