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Specifications
- Antibody Type:Primary
- Antigen Symbol:GCS1
- Clonality:Polyclonal
- Conjugation:Unconjugated
- Host:Rabbit
- ImmunoChemistry:Yes
- Isotype:IgG
- Reactivity:Human,Rat,Mouse
- Western Blot:Yes
- Size:100 µg
- Environmentally Preferable:
- Cross Adsorption:No
- Form:liquid
- Gene ID:7841
- Antigen Synonyms:Mannosyl oligosaccharide glucosidase|MOGS_HUMAN|Processing A glucosidase I|Mannosyl-oligosaccharide glucosidase|Processing A-glucosidase I.|glucosidase I|Mogs|EC 3.2.1.106
- Storage Buffer:Aqueous buffered solution containing 100ug/ml BSA, 50% glycerol and 0.09% sodium azide. Store at -20°C for 12 months.
- Storage Temperature:Store at –20 °C for 12 months
- Concentration:1 μg/μl
- Shipping Temperature:4°C
- Purification:Purified by Protein A
- Cat. No.:10295-990
- Supplier no.:BS-13322R
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: Western Blot: 1:100-1000; IHC-P: 1:100-500; IF(IHC-P): 1:50-200
Type: Primary
Antigen: GCS1
Clonality: Polyclonal
Clone:
Conjugation: Unconjugated
Epitope:
Host: Rabbit
Isotype: IgG
Reactivity: Human, Mouse, Rat