InterPro domain: IPR039698

This entry represents a group of polyprenol reductases, including SRD5A3 from humans, PPRD1 from Arabidopsis and Dfg10 from budding yeasts. SRD5A3 is required for converting polyprenol to dolichol [ 1 ]. Mutations in the SRD5A3 gene cause congenital disorder of glycosylation 1Q, a genetic disease with an extremely broad spectrum of clinical presentations due to defective glycosylation of glycoproteins and glycolipids [ 2 , 2 ]. PPRD2 deficiency results in aberrant development of the male gametophyte and sporophyte [ 3 ]. Homologues are known from animals, plants and fungi.

1. SRD5A3 is required for converting polyprenol to dolichol and is mutated in a congenital glycosylation disorder. Cell 142, 203-17
2. SRD5A3-CDG: a patient with a novel mutation. Eur. J. Paediatr. Neurol. 16, 554-6
3. POLYPRENOL REDUCTASE2 Deficiency Is Lethal in Arabidopsis Due to Male Sterility. Plant Cell 27, 3336-53