Biology:DPM2

Short description: Protein-coding gene in the species Homo sapiens

Generic protein structure example

Dolichol phosphate-mannose biosynthesis regulatory protein is a protein that in humans is encoded by the DPM2 gene.

Function

Dolichol-phosphate mannose (Dol-P-Man) serves as a donor of mannosyl residues on the lumenal side of the endoplasmic reticulum (ER). Lack of Dol-P-Man results in defective surface expression of GPI-anchored proteins, defective N-linked glycosylation and deficient O-mannosylation of α-dystroglycan. Dol-P-Man is synthesized from GDP-mannose and dolichol-phosphate on the cytosolic side of the ER by the enzyme dolichyl-phosphate mannosyltransferase. The protein encoded by this gene is a hydrophobic protein that contains 2 predicted transmembrane domains and a putative ER localization signal near the C-terminus. This protein associates with DPM1 in vivo and is required for the ER localization and stable expression of DPM1 and also enhances the binding of dolichol-phosphate to DPM1.

Clinical significance

Mutations in this gene are associated with congenital disorder of glycosylation.

Further reading

• "Protein-protein interaction panel using mouse full-length cDNAs.". Genome Res. 11 (10): 1758–65. 2001. doi:10.1101/gr.180101. PMID 11591653. 
• "Human dolichol-phosphate-mannose synthase consists of three subunits, DPM1, DPM2 and DPM3.". EMBO J. 19 (11): 2475–82. 2000. doi:10.1093/emboj/19.11.2475. PMID 10835346. 
• "Initial enzyme for glycosylphosphatidylinositol biosynthesis requires PIG-P and is regulated by DPM2.". EMBO J. 19 (16): 4402–11. 2000. doi:10.1093/emboj/19.16.4402. PMID 10944123. 
• "Dissecting and manipulating the pathway for glycosylphos-phatidylinositol-anchor biosynthesis.". Curr Opin Chem Biol 4 (6): 632–8. 2000. doi:10.1016/s1367-5931(00)00151-4. PMID 11102867. 
• "DPM1, the catalytic subunit of dolichol-phosphate mannose synthase, is tethered to and stabilized on the endoplasmic reticulum membrane by DPM3.". J. Biol. Chem. 281 (2): 896–904. 2006. doi:10.1074/jbc.M511311200. PMID 16280320. 
• "Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences.". Proc. Natl. Acad. Sci. U.S.A. 99 (26): 16899–903. 2002. doi:10.1073/pnas.242603899. PMID 12477932. Bibcode: 2002PNAS...9916899M. 
• "The I.M.A.G.E. Consortium: an integrated molecular analysis of genomes and their expression.". Genomics 33 (1): 151–2. 1996. doi:10.1006/geno.1996.0177. PMID 8617505. 
• "DPM2 regulates biosynthesis of dolichol phosphate-mannose in mammalian cells: correct subcellular localization and stabilization of DPM1, and binding of dolichol phosphate.". EMBO J. 17 (17): 4920–9. 1998. doi:10.1093/emboj/17.17.4920. PMID 9724629. 

This article incorporates text from the United States National Library of Medicine, which is in the public domain.