PMVK

PMVK (EC 2.7.4.2) is a peroxisomal enzyme that catalyzes the conversion of mevalonate 5-phosphate into mevalonate 5-diphosphate as the fifth reaction of the cholesterol biosynthetic pathway.[supplied by OMIM

Full Name

phosphomevalonate kinase

Function

Catalyzes the reversible ATP-dependent phosphorylation of mevalonate 5-phosphate to produce mevalonate diphosphate and ADP, a key step in the mevalonic acid mediated biosynthesis of isopentenyl diphosphate and other polyisoprenoid metabolites.

Biological Process

Cholesterol biosynthetic processManual Assertion Based On ExperimentIDA:UniProtKB
Isopentenyl diphosphate biosynthetic process, mevalonate pathwayManual Assertion Based On ExperimentIBA:GO_Central
PhosphorylationIEA:UniProtKB-KW
Response to cholesterolManual Assertion Based On ExperimentIEP:UniProtKB
Sterol biosynthetic processManual Assertion Based On ExperimentIDA:UniProtKB

Cellular Location

Cytoplasm, cytosol

Involvement in disease

Porokeratosis 1, multiple types (POROK1):
A form of porokeratosis, a disorder of faulty keratinization characterized by one or more atrophic patches surrounded by a distinctive hyperkeratotic ridgelike border called the cornoid lamella. The keratotic lesions can progress to overt cutaneous neoplasms, typically squamous cell carcinomas. Multiple clinical variants of porokeratosis are recognized, including porokeratosis of Mibelli, linear porokeratosis, disseminated superficial actinic porokeratosis, palmoplantar porokeratosis, and punctate porokeratosis. Different clinical presentations can be observed among members of the same family. Individuals expressing more than one variant have also been reported.