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Literature summary for 2.7.1.36 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)

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

Protein Variants

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

Inhibitors

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

KM Value [mM]

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

Localization

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

Molecular Weight [Da]

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

Organism

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

Substrates and Products (Substrate)

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

Subunits

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

Ki Value [mM]

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