Anti-MOGS Rabbit Polyclonal Antibody (Cy3®)

Lieferant: Bioss
BS-13322R-CY3
BOSSBS-13322R-CY3EA 900 EUR
BOSSBS-13322R-CY3
Anti-MOGS Rabbit Polyclonal Antibody (Cy3®)
Antikörper
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: Cy3®
Epitope:
Host: Rabbit
Isotype: IgG
Reactivity: Human, Mouse, Rat
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