Specifications
- Antibody type:Primary
- Antigen name:Mannosyl-Oligosaccharide Glucosidase
- Antigen symbol:MOGS
- Clonality:Polyclonal
- Conjugation:Alexa Fluor® 647
- Host:Rabbit
- ImmunoChemistry:Yes
- Isotype:IgG
- Reactivity:Human,Rat,Mouse
- Cross adsorption:No
- Form:liquid
- Gene ID:7841
- Antigen synonyms:CDG2B|GCS1|CWH41|DER7
- Storage buffer:Aqueous buffered solution containing 100ug/ml BSA, 50% glycerol and 0.09% sodium azide. Store at 4°C for 12 months.
- Storage temperature:Store at 4°C for 12 months
- Concentration:1 μg/μl
- Shipping temperature:4°C
- Purification:Purified by Protein A
- Pk:100 µl
Specifications
About this item
Glycosylation of asparagine residues in Asn-X-Ser/Thr motifs in proteins commonly occur in the lumen of the endoplasmic reticulum (ER). Glucosidase I catalyzes the first step in the N-linked oligosaccharide processing pathway. It specifically removes the distal alpha 1,2-linked glucose residue from the Glc3-Man9-GlcNAc2 oligosaccharide precursor. Glucosidase I contains a short cytosolic tail, a single pass transmembrane domain and a large C-terminal catalytic domain located on the luminal side of the ER. Mutations in the gene encoding Glucosidase I result in the congenital disorder glycosylation (CDG-IIb), which is characterized by generalized hypotonia, dysmorphic features, hepatomegaly, hypoventilation, feeding problems, seizures and death. Two point mutations in the Glucosidase I gene have been identified and result in amino acid substitutions, namely Arg486Thr and Phe652Leu, that affect polypeptide folding and active site formation.
Recommended Dilutions: IF(IHC-P): 1:50-200
Type: Primary
Antigen: MOGS
Clonality: Polyclonal
Clone:
Conjugation: Alexa Fluor® 647
Epitope:
Host: Rabbit
Isotype: IgG
Reactivity: Human, Mouse, Rat