YRP13327-01 [Polyclonal Antibody]
GCS1 Rabbit Polyclonal Antibody
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Species:   Rabbit
Applications:   WB IHC IF
Immunogen Range:   KLH conjugated synthetic peptide derived from human GCS1
Clonality:   Polyclonal Antibody
Isotype:   IgG
GENE ID:   7841
Swiss Prot:  
Synonyms:   EC 3.2.1.106, glucosidase I, Mannosyl oligosaccharide glucosidase, Mannosyl-oligosaccharide glucosidase, Mogs, MOGS_HUMAN, Processing A glucosidase I, Processing A-glucosidase I.
Purification:   Purified by Protein A.
Storage:   Aqueous buffered solution containing 100ug/ml BSA, 50% glycerol and 0.09% sodium azide. Store at -20℃ for 12 months
Background:   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.
Caculated MW:   /
Observed MW:   Refer to Figures
Applications:   WB 1:100-1:1000
IHC 1:100-1:500
IF 1:50-1:200
Reacitivity:   Human, Mouse, Rat
For research use only. Not intended for diagnostic or therapeutic use!
Additional information