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Western blot analysis of anti-GCS1 Pab in mouse liver cell line lysate (35ug/lane)
Western blot analysis of anti-GCS1 Pab in mouse liver cell line lysate (35ug/lane)

GCS1 Antibody

CATALOG NUMBER: 61-369

Clonality:
Polyclonal
Tested Applications:
WB
Host Species:
Rabbit
Species Reactivity:
Human, Mouse
Conjugate:
Unconjugated
Specifications
Host Species:
Rabbit
Species Reactivity:
Human, Mouse
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.
Conjugate:
Unconjugated
Tested Applications:
WB
Application Note:
For WB starting dilution is: 1:1000
Predicted Molecular Weight:
92 kDa
Purification:
This antibody is prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis
Clonality:
Polyclonal
Isotype:
Rabbit Ig
Physical State:
Liquid
Buffer:
Supplied in PBS with 0.09% (W/V) sodium azide.
Concentration:
batch dependent
Storage Conditions:
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.
Ncbi Official Symbol:
MOGS
Additional Names:
Mannosyl-oligosaccharide glucosidase, Processing A-glucosidase I, MOGS, GCS1
Protein Accession Number:
Q13724
Protein Gi Number:
116242490
Ncbi Gene Id Number:
7841
User Note:
Optimal dilutions for each application to be determined by the researcher.
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 Reference 1:
Volker, C., et al., Glycobiology 12(8):473-483 (2002).
Background Reference 2:
De Praeter, C.M., et al., Am. J. Hum. Genet. 66(6):1744-1756 (2000).
Background Reference 3:
Kalz-Fuller, B., et al., Eur. J. Biochem. 231(2):344-351 (1995).
Background Reference 4:
Kalz-Fueller, B., et al., Eur. J. Biochem. 249, 912-912 (1997).

FOR RESEARCH USE ONLY

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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.

CATALOG NUMBER:

61-369

List Size:
400 ul

List Price:

$495.00

Datasheet
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