In enzymology, a dolichol kinase () is an enzyme that catalyzes the chemical reaction

CTP + dolichol CDP + dolichyl phosphate

Thus, the two substrates of this enzyme are CTP and dolichol, whereas its two products are CDP and dolichyl phosphate.

Dolichol kinase

This enzyme belongs to the family of transferases, to be specific, those transferring phosphorus-containing groups (phosphotransferases) with an alcohol group as acceptor. The systematic name of this enzyme class is CTP:dolichol O-phosphotransferase. This enzyme is also called dolichol phosphokinase. This enzyme participates in N-glycan biosynthesis.

Dolichol kinase

In humans dolichol kinase is encoded by the DOLK gene.

Dolichol kinase

Function

Dolichyl monophosphate is an essential glycosyl carrier lipid for C- and O-mannosylation and N-glycosylation of proteins and for biosynthesis of glycosylphosphatidylinositol anchors in endoplasmic reticulum (ER). Dolichol kinase catalyzes CTP-mediated phosphorylation of dolichol, the terminal step in de novo dolichyl monophosphate biosynthesis.

Clinical significance

Mutations in DOLK cause a subtype of the congenital disorders of glycosylation, DOLK-CDG (CDG-Im).

See also

Dolichol kinase deficiency

References

Further reading

External links

GeneReviews/NCBI/NIH/UW entry on Congenital Disorders of Glycosylation Overview

EC 2.7.1

Enzymes of unknown structure