GCS1 Antibody
| Code | Size | Price |
|---|
| PSI-61-369-400ul | 400ul | £626.00 |
Quantity:
Prices exclude any Taxes / VAT
Overview
Host Type: Rabbit
Antibody Isotype: Rabbit Ig
Antibody Clonality: Polyclonal
Regulatory Status: RUO
Target Species:
- Human
- Mouse
Application: Western Blot (WB)
Storage:
Store at 4˚C for three months and -20˚C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.
Documents
Further Information
Additional Names:
Mannosyl-oligosaccharide glucosidase, Processing A-glucosidase I, MOGS, GCS1
Application Note:
For WB starting dilution is: 1:1000
Background:
GCS1 cleaves the distal alpha 1,2-linked glucose residue from the Glc(3)Man(9)GlcNAc(2) oligosaccharide precursor in a highly specific manner. Defects in GCS1 are the cause of type IIb congenital disorder of glycosylation (CDGIIb). This syndrome is also known as glucosidase I deficiency and is characterized by marked generalized hypotonia and hypomotility of the neonate, dysmorphic features, including a prominent occiput, short palpebral fissures, retrognathia, high arched palate, generalized edema, and hypoplastic genitalia. Symptoms include hepatomegaly, hypoventilation, feeding problems and seizures. The clinical course is progressive and survival is at most a few months.
Background References:
- Volker, C., et al., Glycobiology 12(8):473-483 (2002).
- De Praeter, C.M., et al., Am. J. Hum. Genet. 66(6):1744-1756 (2000).
- Kalz-Fuller, B., et al., Eur. J. Biochem. 231(2):344-351 (1995).
- Kalz-Fueller, B., et al., Eur. J. Biochem. 249, 912-912 (1997).
Buffer:
Supplied in PBS with 0.09% (W/V) sodium azide.
Concentration:
batch dependent
Conjugate:
Unconjugated
DISCLAIMER:
Optimal dilutions/concentrations should be determined by the end user. The information provided is a guideline for product use. This product is for research use only.
Immunogen:
This GCS1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 796-826 amino acids from the C-terminal region of human GCS1.
NCBI Gene ID #:
7841
NCBI Official Name:
Mannosyl-oligosaccharide glucosidase
NCBI Official Symbol:
MOGS
NCBI Organism:
Homo sapiens
Physical State:
Liquid
PREDICTED MOLECULAR WEIGHT:
92 kDa
Protein Accession #:
Q13724
Protein GI Number:
116242490
Purification:
This antibody is prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis
Swissprot #:
Q13724
User NOte:
Optimal dilutions for each application to be determined by the researcher.