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Literature summary extracted from

  • Houten, S.M.; Wanders, R.J.; Waterham, H.R.
    Biochemical and genetic aspects of mevalonate kinase and its deficiency (2000), Biochim. Biophys. Acta, 1529, 19-32.
    View publication on PubMed

Cloned(Commentary)

EC Number Cloned (Comment) Organism
2.7.1.36 expression of wild-type and N301T mutant mevalonate kinase in COS-7 cells Homo sapiens

Protein Variants

EC Number Protein Variants Comment Organism
2.7.1.36 A148T mutation may be responsible for the hyperimmunoglobulinemia phenotype Homo sapiens
2.7.1.36 H20P markedly decreased mevalonate kinase activity when expressed in Escherichia coli Homo sapiens
2.7.1.36 I268T markedly decreased mevalonate kinase activity when expressed in Escherichia coli Homo sapiens
2.7.1.36 L264F markedly decreased mevalonate kinase activity when expressed in Escherichia coli Homo sapiens
2.7.1.36 L265P markedly decreased mevalonate kinase activity when expressed in Escherichia coli Homo sapiens
2.7.1.36 N301T 5-20% of wild-type activity Homo sapiens
2.7.1.36 P165L mutation may be responsible for the hyperimmunoglobulinemia phenotype Homo sapiens
2.7.1.36 T243I markedly decreased mevalonate kinase activity when expressed in Escherichia coli Homo sapiens
2.7.1.36 V310M markedly decreased mevalonate kinase activity when expressed in Escherichia coli Homo sapiens
2.7.1.36 V377I mutation may be responsible for the hyperimmunoglobulinemia phenotype Homo sapiens

Inhibitors

EC Number Inhibitors Comment Organism Structure
2.7.1.36 farnesyl diphosphate
-
 Rattus norvegicus
2.7.1.36 farnesyl diphosphate
-
 Sus scrofa
2.7.1.36 geranyl diphosphate
-
 Homo sapiens
2.7.1.36 geranyl diphosphate
-
 Sus scrofa

KM Value [mM]

EC Number KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
2.7.1.36 0.019
-
 (RS)mevalonate liver mevalonate kinase Sus scrofa
2.7.1.36 0.069
-
 (RS)mevalonate
-
 Methanocaldococcus jannaschii
2.7.1.36 0.069
-
 (RS)mevalonate recombinant mevalonate kinase Sus scrofa
2.7.1.36 0.092
-
 ATP recombinant mevalonate kinase Sus scrofa
2.7.1.36 0.27
-
 (RS)mevalonate liver mevalonate kinase Rattus norvegicus
2.7.1.36 0.3
-
 ATP liver mevalonate kinase Sus scrofa
2.7.1.36 1.75
-
 ATP liver mevalonate kinase Rattus norvegicus

Localization

EC Number Localization Comment Organism GeneOntology No. Textmining
2.7.1.36 cytosol
-
 Sus scrofa 5829
-
2.7.1.36 cytosol
-
 Rattus norvegicus 5829
-
2.7.1.36 peroxisome mevalonate kinase is predominantly localized in peroxisomes, but is easily solubilized and released into the cytosol Rattus norvegicus 5777
-

Molecular Weight [Da]

EC Number Molecular Weight [Da] Molecular Weight Maximum [Da] Comment Organism
2.7.1.36 41990
-
 2 * 41990, deduced from nucleotide sequence Rattus norvegicus

Organism

EC Number Organism UniProt Comment Textmining
2.7.1.36 Homo sapiens Q03426 mevalonate kinase deficiency causes mevalonic aciduria, hyperimmunoglobuinemia D and periodic fever syndrome
-
2.7.1.36 Methanocaldococcus jannaschii
-
-
-
2.7.1.36 Rattus norvegicus P17256
-
-
2.7.1.36 Sus scrofa
-
-
-

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
2.7.1.36 ATP + mevalonate
-
 Methanocaldococcus jannaschii ADP + phosphomevalonate
-
 ?
2.7.1.36 ATP + mevalonate
-
 Rattus norvegicus ADP + phosphomevalonate
-
 ?

Subunits

EC Number Subunits Comment Organism
2.7.1.36 dimer
-
 Sus scrofa
2.7.1.36 dimer
-
 Methanocaldococcus jannaschii
2.7.1.36 dimer 2 * 41990, deduced from nucleotide sequence Rattus norvegicus

Ki Value [mM]

EC Number Ki Value [mM] Ki Value maximum [mM] Inhibitor Comment Organism Structure
2.7.1.36 0.002
-
 farnesyl diphosphate liver mevalonate kinase Sus scrofa
2.7.1.36 0.002
-
 geranyl diphosphate liver mevalonate kinase Sus scrofa
2.7.1.36 0.0025
-
 farnesyl diphosphate
-
 Rattus norvegicus
2.7.1.36 0.116
-
 geranyl diphosphate
-
 Homo sapiens