ALG6 Antibody (Z164): sc-100506

ALG6 Antibody (Z164) is a mouse monoclonal IgG2a kappa light chain antibody that detects ALG6 protein of mouse, rat, and human origin by western blotting (WB), immunoprecipitation (IP), and enzyme-linked immunosorbent assay (ELISA). Anti-ALG6 antibody (Z164) is available as a non-conjugated format. ALG6, also known as asparagine-linked glycosylation 6 homolog, is a crucial 507 amino acid member of the ALG6/ALG8 glucosyltransferase family, functioning as an α1,3-glucosyltransferase essential for proper asparagine-linked glycosylation of proteins. ALG6 is primarily located in the endoplasmic reticulum (ER), where ALG6 plays a vital role in the initial step of N-linked glycosylation by adding the first of three glucose residues to dolichylpyrophosphate-linked oligosaccharides. ALG6′s location in the ER ensures correct folding and stability of glycoproteins, which are critical for various cellular functions. Disruptions in the gene encoding ALG6 can lead to severe consequences, such as congenital disorder of glycosylation type 1C (CDG1C), a multisystem disease characterized by under-glycosylated serum proteins, delayed statomotor development, mental retardation, and muscular hypotonia in affected patients.

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ALG6 Antibody (Z164) References:

1. A mutation in the human ortholog of the Saccharomyces cerevisiae ALG6 gene causes carbohydrate-deficient glycoprotein syndrome type-Ic.  |  Imbach, T., et al. 1999. Proc Natl Acad Sci U S A. 96: 6982-7. PMID: 10359825
2. Analysis of multiple mutations in the hALG6 gene in a patient with congenital disorder of glycosylation Ic.  |  Westphal, V., et al. 2000. Mol Genet Metab. 70: 219-23. PMID: 10924277
3. Reduced heparan sulfate accumulation in enterocytes contributes to protein-losing enteropathy in a congenital disorder of glycosylation.  |  Westphal, V., et al. 2000. Am J Pathol. 157: 1917-25. PMID: 11106564
4. Balancing N-linked glycosylation to avoid disease.  |  Freeze, HH. and Westphal, V. 2001. Biochimie. 83: 791-9. PMID: 11530212
5. Common origin and evolution of glycosyltransferases using Dol-P-monosaccharides as donor substrate.  |  Oriol, R., et al. 2002. Mol Biol Evol. 19: 1451-63. PMID: 12200473
6. Congenital disorder of glycosylation Ic in patients of Indian origin.  |  Newell, JW., et al. 2003. Mol Genet Metab. 79: 221-8. PMID: 12855228
7. Identification of a frequent variant in ALG6, the cause of Congenital Disorder of Glycosylation-Ic.  |  Westphal, V., et al. 2003. Hum Mutat. 22: 420-1. PMID: 14517965
8. Clinical and molecular characterization of the first adult congenital disorder of glycosylation (CDG) type Ic patient.  |  Sun, L., et al. 2005. Am J Med Genet A. 137: 22-6. PMID: 16007612
9. Effects of N-glycosylation and inositol on the ER stress response in yeast Saccharomyces cerevisiae.  |  Uchimura, S., et al. 2005. Biosci Biotechnol Biochem. 69: 1274-80. PMID: 16041130