Chitobiosyldiphosphodolichol beta-mannosyltransferase ALG1-like (IPR026051)

Short name: ALG1-like

Family relationships



ALG1 (Asparagine-linked glycosylation protein 1) proteins participate in the formation of the lipid-linked precursor oligosaccharide for N-glycosylation. They are also involved in assembling the dolichol-pyrophosphate-GlcNAc(2)-Man5 intermediate on the cytoplasmic surface of the ER [PMID: 10704531, PMID: 15044395].

Defects in human ALG1 are the cause of congenital disorder of glycosylation type 1K (CDG1K). CDGs are characterised 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. [PMID: 14973778, PMID: 14973782, PMID: 14709599].

Glycosylation and growth of Alg1-deficient PRY56 yeast cells, showing a temperature-sensitive phenotype, could be restored by the human wild-type allele [PMID: 14973782].

GO terms

Biological Process

No terms assigned in this category.

Molecular Function

GO:0016757 transferase activity, transferring glycosyl groups

Cellular Component

No terms assigned in this category.

Contributing signatures

Signatures from InterPro member databases are used to construct an entry.