ALG8 blocking peptide

ALG8 Antibody (N-term) Blocking Peptide

• Gene Names: ALG8; CDG1H
• Synonyms: ALG8; ALG8 Antibody (N-term) Blocking Peptide; Probable dolichyl pyrophosphate Glc1Man9GlcNAc2 alpha-1;3-glucosyltransferase; Asparagine-linked glycosylation protein 8 homolog; Dol-P-Glc:Glc(1)Man(9)GlcNAc(2)-PP-dolichyl alpha-1;3-glucosyltransferase; Dolichyl-P-Glc:Glc1Man9GlcNAc2-PP-dolichyl glucosyltransferase; ALG8 blocking peptide

• Form/Format: Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.
• Sequence Length: 526

• Cellular Location: Endoplasmic reticulum membrane; Multi-pass membrane protein
• Preparation and Storage: Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C.

Related Product Information for ALG8 blocking peptide

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 (By similarity).

NCBI and Uniprot Product Information

• NCBI GI #: 143811361
• NCBI GeneID: 79053
• UniProt Accession #: Q9BVK2; O60860; A6NDW6
• Molecular Weight: 53,181 Da
• NCBI Official Full Name: Probable dolichyl pyrophosphate Glc1Man9GlcNAc2 alpha-1,3-glucosyltransferase
• NCBI Official Synonym Full Names: ALG8, alpha-1,3-glucosyltransferase
• NCBI Official Symbol: ALG8
• NCBI Official Synonym Symbols: CDG1H
• NCBI Protein Information: probable dolichyl pyrophosphate Glc1Man9GlcNAc2 alpha-1,3-glucosyltransferase
• UniProt Protein Name: Probable dolichyl pyrophosphate Glc1Man9GlcNAc2 alpha-1,3-glucosyltransferase
• Protein Family: Glycosyltransferase
• UniProt Gene Name: ALG8
• UniProt Entry Name: ALG8_HUMAN

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: Transferase; Membrane protein, multi-pass; EC 2.4.1.265; Membrane protein, integral; Glycan Metabolism - N-glycan biosynthesis

Chromosomal Location of Human Ortholog: 11q14.1

Cellular Component: endoplasmic reticulum membrane

Molecular Function: alpha-1,3-mannosyltransferase activity; dolichyl pyrophosphate Man9GlcNAc2 alpha-1,3-glucosyltransferase activity; dolichyl-phosphate-glucose-glycolipid alpha-glucosyltransferase activity

Biological Process: dolichol-linked oligosaccharide biosynthetic process; oligosaccharide-lipid intermediate assembly; protein amino acid N-linked glycosylation

Disease: Congenital Disorder Of Glycosylation, Type Ih