Glucosidase I is the first enzyme in the N-linked oligosaccharide processing pathway. GCS1 cleaves the distal alpha-1,2-linked glucose residue from the Glc(3)-Man(9)-GlcNAc(2) oligosaccharide precursor. GCS1 is located in the lumen of the endoplasmic reticulum.GCS1 may also refer to "generative cell specific 1", also called HAP2 (hapless2), a gene of lower eukaryotes which is thought to be responsible for gametes fusion .
De Praeter et al. (2000) identified a glucosidase I defect in a neonate with severe generalized hypotonia and dysmorphic features consistent with congenital disorder of glycosylation type IIb (CDG2B). Molecular studies showed that the patient was a compound heterozygote for 2 missense mutations in the GCS1 gene.
Organism species: Homo sapiens (Human)
CATALOG NO. | PRODUCT NAME | APPLICATIONS | |
Proteins | RPD185Hu01 | Recombinant Mannosyl Oligosaccharide Glucosidase (MOGS) | Positive Control; Immunogen; SDS-PAGE; WB. |
Antibodies | PAD185Hu01 | Polyclonal Antibody to Mannosyl Oligosaccharide Glucosidase (MOGS) | WB; IHC; ICC; IP. |
Assay Kits | n/a | CLIA Kit for Mannosyl Oligosaccharide Glucosidase (MOGS) | CLIA Kit Customized Service Offer |
n/a | ELISA Kit for Mannosyl Oligosaccharide Glucosidase (MOGS) | ELISA Kit Customized Service Offer |
Organism species: Mus musculus (Mouse)
CATALOG NO. | PRODUCT NAME | APPLICATIONS | |
Proteins | RPD185Mu01 | Recombinant Mannosyl Oligosaccharide Glucosidase (MOGS) | Positive Control; Immunogen; SDS-PAGE; WB. |
Antibodies | PAD185Mu01 | Polyclonal Antibody to Mannosyl Oligosaccharide Glucosidase (MOGS) | WB; IHC; ICC; IP. |
Assay Kits | n/a | CLIA Kit for Mannosyl Oligosaccharide Glucosidase (MOGS) | CLIA Kit Customized Service Offer |
n/a | ELISA Kit for Mannosyl Oligosaccharide Glucosidase (MOGS) | ELISA Kit Customized Service Offer |
Organism species: Rattus norvegicus (Rat)
CATALOG NO. | PRODUCT NAME | APPLICATIONS | |
Proteins | n/a | Recombinant Mannosyl Oligosaccharide Glucosidase (MOGS) | Recombinant Protein Customized Service Offer |
Antibodies | n/a | Monoclonal Antibody to Mannosyl Oligosaccharide Glucosidase (MOGS) | Monoclonal Antibody Customized Service Offer |
n/a | Polyclonal Antibody to Mannosyl Oligosaccharide Glucosidase (MOGS) | Polyclonal Antibody Customized Service Offer | |
Assay Kits | n/a | CLIA Kit for Mannosyl Oligosaccharide Glucosidase (MOGS) | CLIA Kit Customized Service Offer |
n/a | ELISA Kit for Mannosyl Oligosaccharide Glucosidase (MOGS) | ELISA Kit Customized Service Offer |
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