DPM2 explained

Dolichol phosphate-mannose biosynthesis regulatory protein is a protein that in humans is encoded by the DPM2 gene.^[1]

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.^[1]

Clinical significance

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

Further reading

• Suzuki H, Fukunishi Y, Kagawa I . Protein-protein interaction panel using mouse full-length cDNAs. . Genome Res. . 11 . 10 . 1758–65 . 2001 . 11591653 . 10.1101/gr.180101 . 311163. etal.
• Maeda Y, Tanaka S, Hino J . Human dolichol-phosphate-mannose synthase consists of three subunits, DPM1, DPM2 and DPM3. . EMBO J. . 19 . 11 . 2475–82 . 2000 . 10835346 . 10.1093/emboj/19.11.2475 . 212771. etal.
• Watanabe R, Murakami Y, Marmor MD . Initial enzyme for glycosylphosphatidylinositol biosynthesis requires PIG-P and is regulated by DPM2. . EMBO J. . 19 . 16 . 4402–11 . 2000 . 10944123 . 10.1093/emboj/19.16.4402 . 302040. etal.
• Kinoshita T, Inoue N . Dissecting and manipulating the pathway for glycosylphos-phatidylinositol-anchor biosynthesis. . Curr Opin Chem Biol . 4 . 6 . 632–8 . 2000 . 11102867 . 10.1016/s1367-5931(00)00151-4.
• Ashida H, Maeda Y, Kinoshita T . 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 . 16280320 . 10.1074/jbc.M511311200 . free .
• Strausberg RL, Feingold EA, Grouse LH . 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 . 12477932 . 10.1073/pnas.242603899 . 139241. etal. 2002PNAS...9916899M . free .
• Lennon G, Auffray C, Polymeropoulos M, Soares MB . The I.M.A.G.E. Consortium: an integrated molecular analysis of genomes and their expression. . Genomics . 33 . 1 . 151–2 . 1996 . 8617505 . 10.1006/geno.1996.0177 . free .
• Maeda Y, Tomita S, Watanabe R . 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 . 9724629 . 10.1093/emboj/17.17.4920 . 1170821. etal.

Notes and References

1. Web site: Entrez Gene: dolichyl-phosphate mannosyltransferase polypeptide 2.