Western Blot (WB)
(ALG8 Antibody (N-term) western blot analysis in MDA-MB453 cell line lysates (35ug/lane).This demonstrates the ALG8 antibody detected the ALG8 protein (arrow).)
Rabbit ALG8 Polyclonal Antibody | anti-ALG8 antibody
ALG8 Antibody (N-term)
Western Blot (WB)
(ALG8 Antibody (N-term) western blot analysis in MDA-MB453 cell line lysates (35ug/lane).This demonstrates the ALG8 antibody detected the ALG8 protein (arrow).)
Western Blot (WB)
(ALG8 Antibody (N-term) western blot analysis in MDA-MB453 cell line lysates (35ug/lane).This demonstrates the ALG8 antibody detected the ALG8 protein (arrow).)
glucosyltransferase family. The encoded protein catalyzes the
addition of the second glucose residue to the lipid-linked
oligosaccharide precursor for N-linked glycosylation of proteins.
Mutations in this gene have been associated with congenital
disorder of glycosylation type Ih (CDG-Ih). Alternatively spliced
transcript variants encoding different isoforms have been
identified.
Stolting, T., et al. Mol. Genet. Metab. 98(3):305-309(2009)
Jaeken, J., et al. Curr. Opin. Pediatr. 16(4):434-439(2004)
Schollen, E., et al. J. Med. Genet. 41(7):550-556(2004)
Jaeken, J. J. Inherit. Metab. Dis. 27(3):423-426(2004)
NCBI and Uniprot Product Information
NCBI Description
This gene encodes a member of the ALG6/ALG8 glucosyltransferase family. The encoded protein catalyzes the addition of the second glucose residue to the lipid-linked oligosaccharide precursor for N-linked glycosylation of proteins. Mutations in this gene have been associated with congenital disorder of glycosylation type Ih (CDG-Ih). Alternatively spliced transcript variants encoding different isoforms have been identified. [provided by RefSeq, Jul 2008]
Uniprot Description
ALG8: Adds the second glucose residue to the lipid-linked oligosaccharide precursor for N-linked glycosylation. Transfers glucose from dolichyl phosphate glucose (Dol-P-Glc) onto the lipid-linked oligosaccharide Glc(1)Man(9)GlcNAc(2)-PP-Dol. Defects in ALG8 are the cause of congenital disorder of glycosylation type 1H (CDG1H). CDGs are a family of severe inherited diseases caused by a defect in protein N- glycosylation. They are characterized by under-glycosylated serum proteins. These multisystem disorders present with a wide variety of clinical features, such as disorders of the nervous system development, psychomotor retardation, dysmorphic features, hypotonia, coagulation disorders, and immunodeficiency. The broad spectrum of features reflects the critical role of N-glycoproteins during embryonic development, differentiation, and maintenance of cell functions. Belongs to the ALG6/ALG8 glucosyltransferase family.
Protein type: Glycan Metabolism - N-glycan biosynthesis; Membrane protein, multi-pass; Transferase; EC 2.4.1.265; Membrane protein, integral
Chromosomal Location of Human Ortholog: 11q14.1
Cellular Component: endoplasmic reticulum membrane; integral to membrane
Molecular Function: dolichyl pyrophosphate Man9GlcNAc2 alpha-1,3-glucosyltransferase activity; alpha-1,3-mannosyltransferase activity
Biological Process: cellular protein metabolic process; dolichol-linked oligosaccharide biosynthetic process; protein amino acid N-linked glycosylation; oligosaccharide-lipid intermediate assembly; protein amino acid N-linked glycosylation via asparagine; post-translational protein modification
Disease: Congenital Disorder Of Glycosylation, Type Ih
Research Articles on ALG8
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Product Notes
The ALG8 alg8 (Catalog #AAA9210308) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The immunogen sequence is 15-41. The ALG8 Antibody (N-term) reacts with Human (Predicted Reactivity: Bovine, Mouse) and may cross-react with other species as described in the data sheet. AAA Biotech's ALG8 can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB), ELISA (EIA). WB~~1:1000. Researchers should empirically determine the suitability of the ALG8 alg8 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "ALG8, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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