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TMEM15

MIM.610746 9q34.11

TMEM15 gene (MIM.610746) encodes the dolichol kinase (DK1), an enzyme responsible for the final step of the de novo biosynthesis of dolichol phosphate.

Pathology

- congenital disorder of glycosylation type Im (CDG1M), also known as dolichol kinase deficiency (DK1 deficiency), can be caused by mutation in the TMEM15 gene.

References

- Kranz C, Jungeblut C, Denecke J, Erlekotte A, Sohlbach C, Debus V, Kehl HG, Harms E, Reith A, Reichel S, Grobe H, Hammersen G, Schwarzer U, Marquardt T. A defect in dolichol phosphate biosynthesis causes a new inherited disorder with death in early infancy. Am J Hum Genet. 2007 Mar;80(3):433-40. PMID: 17273964