GCS1 polyclonal antibody
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More Files
- More Functions
Western Blot (Tissue lysate)
Western blot analysis of GCS1 polyclonal antibody (Cat # PAB4280) in mouse liver tissue lysate (35 ug/lane). GCS1 (arrow) was detected using the purified GCS1 polyclonal antibody (Cat # PAB4280).
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Specification
Product Description
Rabbit polyclonal antibody raised against synthetic peptide of GCS1.
Immunogen
A synthetic peptide (conjugated with KLH) corresponding to C-terminus of human GCS1.
Host
Rabbit
Reactivity
Human, Mouse
Form
Liquid
Purification
Protein G purification
Recommend Usage
ELISA (1:1000)
Western Blot (1:100-500)
The optimal working dilution should be determined by the end user.Storage Buffer
In PBS (0.09% sodium azide)
Storage Instruction
Store at 4°C. For long term storage store at -20°C.
Aliquot to avoid repeated freezing and thawing.Note
This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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Applications
Western Blot (Tissue lysate)
Western blot analysis of GCS1 polyclonal antibody (Cat # PAB4280) in mouse liver tissue lysate (35 ug/lane). GCS1 (arrow) was detected using the purified GCS1 polyclonal antibody (Cat # PAB4280).Enzyme-linked Immunoabsorbent Assay
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Gene Info — GCS1
Entrez GeneID
7841Protein Accession#
GCS1_HUMANGene Name
GCS1
Gene Alias
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Gene Description
glucosidase I
Gene Ontology
HyperlinkGene Summary
This gene encodes the first enzyme in the N-linked oligosaccharide processing pathway. The enzyme cleaves the distal alpha-1,2-linked glucose residue from the Glc(3)-Man(9)-GlcNAc(2) oligosaccharide precursor. This protein is located in the lumen of the endoplasmic reticulum. Defects in this gene are a cause of type IIb congenital disorder of glycosylation (CDGIIb). Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq
Other Designations
mannosyl-oligosaccharide glucosidase|processing A-glucosidase I
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Interactome
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Pathway
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Publication Reference
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A novel disorder caused by defective biosynthesis of N-linked oligosaccharides due to glucosidase I deficiency.
De Praeter CM, Gerwig GJ, Bause E, Nuytinck LK, Vliegenthart JF, Breuer W, Kamerling JP, Espeel MF, Martin JJ, De Paepe AM, Chan NW, Dacremont GA, Van Coster RN.
American Journal of Human Genetics 2000 Jun; 66(6):1744.
Application：WB-Ce, WB-Ti, Human, Liver, Fibroblast cells.
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Cloning and expression of glucosidase I from human hippocampus.
Kalz-Fuller B, Bieberich E, Bause E.
European Journal of Biochemistry 1995 Jul; 231(2):344.
Application：IF, WB-Ti, WB-Tr, Human, Monkey, Pig, COS cells, Human hippocampus, Pig livers.
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A novel disorder caused by defective biosynthesis of N-linked oligosaccharides due to glucosidase I deficiency.
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