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0.007 - 1.55
(R)-mevalonate 5-diphosphate
0.034 - 2.04
(R)-mevalonate 5-phosphate
0.007 - 1.55
(R)-5-diphosphomevalonate
0.034 - 2.04
(R)-5-phosphomevalonate
additional information
additional information
-
0.007
(R)-mevalonate 5-diphosphate
pH 7.0, 30°C, recombinant mutant K48M
0.041
(R)-mevalonate 5-diphosphate
pH 7.0, 30°C, recombinant wild-type enzyme
0.041
(R)-mevalonate 5-diphosphate
pH 7.0, 30°C, recombinant wild-type enzyme
0.041
(R)-mevalonate 5-diphosphate
pH 7.0, 30°C, recombinant mutant R73M
0.044
(R)-mevalonate 5-diphosphate
pH 7.0, 30°C, recombinant mutant K69M
0.045
(R)-mevalonate 5-diphosphate
pH 7.0, 30°C, recombinant mutant R93M
0.047
(R)-mevalonate 5-diphosphate
pH 7.0, 30°C, recombinant mutant R18Q
0.057
(R)-mevalonate 5-diphosphate
pH 7.0, 30°C, recombinant mutant R138M
0.079
(R)-mevalonate 5-diphosphate
pH 7.0, 30°C, recombinant mutant K19M
0.102
(R)-mevalonate 5-diphosphate
pH 7.0, 30°C, recombinant mutant R130M
0.138
(R)-mevalonate 5-diphosphate
pH 7.0, 30°C, recombinant mutant D23N
0.139
(R)-mevalonate 5-diphosphate
pH 7.0, 30°C, recombinant mutant R141M
0.187
(R)-mevalonate 5-diphosphate
pH 7.0, 30°C, recombinant mutant K17M
1.25
(R)-mevalonate 5-diphosphate
pH 7.0, 30°C, recombinant mutant R111M
1.55
(R)-mevalonate 5-diphosphate
pH 7.0, 30°C, recombinant mutant R84M
0.034
(R)-mevalonate 5-phosphate
pH 7.0, 30°C, recombinant wild-type enzyme
0.034
(R)-mevalonate 5-phosphate
pH 7.0, 30°C, recombinant wild-type enzyme
0.047
(R)-mevalonate 5-phosphate
pH 7.0, 30°C, recombinant mutant R138M
0.048
(R)-mevalonate 5-phosphate
pH 7.0, 30°C, recombinant mutant R93M
0.1
(R)-mevalonate 5-phosphate
pH 7.0, 30°C, recombinant mutant R73M
0.102
(R)-mevalonate 5-phosphate
pH 7.0, 30°C, recombinant mutant R130M
0.11
(R)-mevalonate 5-phosphate
pH 7.0, 30°C, recombinant mutant K69M
0.116
(R)-mevalonate 5-phosphate
pH 7.0, 30°C, recombinant mutant D23N
0.123
(R)-mevalonate 5-phosphate
pH 7.0, 30°C, recombinant mutant R18Q
0.131
(R)-mevalonate 5-phosphate
pH 7.0, 30°C, recombinant mutant K48M
0.176
(R)-mevalonate 5-phosphate
pH 7.0, 30°C, recombinant mutant K17M
0.225
(R)-mevalonate 5-phosphate
pH 7.0, 30°C, recombinant mutant R141M
0.23
(R)-mevalonate 5-phosphate
pH 7.0, 30°C, recombinant mutant K19M
1.71
(R)-mevalonate 5-phosphate
pH 7.0, 30°C, recombinant mutant R84M
2.04
(R)-mevalonate 5-phosphate
pH 7.0, 30°C, recombinant mutant R111M
0.047
ADP
pH 7.0, 30°C, recombinant wild-type enzyme
0.047
ADP
pH 7.0, 30°C, recombinant wild-type enzyme
0.06
ADP
pH 7.0, 30°C, recombinant mutant K48M
0.065
ADP
pH 7.0, 30°C, recombinant mutant R18Q
0.079
ADP
pH 7.0, 30°C, recombinant mutant D23N
0.091
ADP
pH 7.0, 30°C, recombinant mutant R93M
0.093
ADP
pH 7.0, 30°C, recombinant mutant R111M
0.106
ADP
pH 7.0, 30°C, recombinant mutant K69M
0.118
ADP
pH 7.0, 30°C, recombinant mutant R73M
0.131
ADP
pH 7.0, 30°C, recombinant mutant K19M
0.164
ADP
pH 7.0, 30°C, recombinant mutant R130M
0.238
ADP
pH 7.0, 30°C, recombinant mutant R84M
0.286
ADP
pH 7.0, 30°C, recombinant mutant K17M
0.451
ADP
pH 7.0, 30°C, recombinant mutant R138M
5.62
ADP
pH 7.0, 30°C, recombinant mutant R141M
0.094
ATP
pH 7.0, 30°C, recombinant mutant R73M
0.101
ATP
pH 7.0, 30°C, recombinant mutant R93M
0.107
ATP
pH 7.0, 30°C, recombinant wild-type enzyme
0.107
ATP
pH 7.0, 30°C, recombinant wild-type enzyme
0.107
ATP
ATP in form of MgATP2-
0.229
ATP
pH 7.0, 30°C, recombinant mutant R130M
0.236
ATP
pH 7.0, 30°C, recombinant mutant K48M
0.268
ATP
pH 7.0, 30°C, recombinant mutant K69M
0.303
ATP
pH 7.0, 30°C, recombinant mutant R84M
0.518
ATP
pH 7.0, 30°C, recombinant mutant R138M
0.536
ATP
pH 7.0, 30°C, recombinant mutant R18Q
0.536
ATP
ATP in form of MgATP2-
0.556
ATP
pH 7.0, 30°C, recombinant mutant K17M
0.556
ATP
ATP in form of MgATP2-
0.792
ATP
pH 7.0, 30°C, recombinant mutant R111M
0.815
ATP
pH 7.0, 30°C, recombinant mutant D23N
0.815
ATP
ATP in form of MgATP2-
1.312
ATP
pH 7.0, 30°C, recombinant mutant K19M
1.312
ATP
ATP in form of MgATP2-
5.2
ATP
pH 7.0, 30°C, recombinant mutant R141M
0.007
(R)-5-diphosphomevalonate
mutant K48M, 30°C, pH 7.0
0.041
(R)-5-diphosphomevalonate
wild-type, 30°C, pH 7.0
0.041
(R)-5-diphosphomevalonate
mutant R73M, 30°C, pH 7.0
0.044
(R)-5-diphosphomevalonate
mutant K69M, 30°C, pH 7.0
0.045
(R)-5-diphosphomevalonate
mutant R93M, 30°C, pH 7.0
0.057
(R)-5-diphosphomevalonate
mutant R138M, 30°C, pH 7.0
0.102
(R)-5-diphosphomevalonate
mutant R130M, 30°C, pH 7.0
0.139
(R)-5-diphosphomevalonate
mutant R141M, 30°C, pH 7.0
1.25
(R)-5-diphosphomevalonate
mutant R111M, 30°C, pH 7.0
1.55
(R)-5-diphosphomevalonate
mutant R84M, 30°C, pH 7.0
0.034
(R)-5-phosphomevalonate
wild-type, 30°C, pH 7.0
0.047
(R)-5-phosphomevalonate
mutant R138M, 30°C, pH 7.0
0.048
(R)-5-phosphomevalonate
mutant R93M, 30°C, pH 7.0
0.1
(R)-5-phosphomevalonate
mutant R73M, 30°C, pH 7.0
0.102
(R)-5-phosphomevalonate
mutant R130M, 30°C, pH 7.0
0.11
(R)-5-phosphomevalonate
mutant K69M, 30°C, pH 7.0
0.131
(R)-5-phosphomevalonate
mutant K48M, 30°C, pH 7.0
0.225
(R)-5-phosphomevalonate
mutant R141M, 30°C, pH 7.0
1.71
(R)-5-phosphomevalonate
mutant R84M, 30°C, pH 7.0
2.04
(R)-5-phosphomevalonate
mutant R111M, 30°C, pH 7.0
0.047
ADP
wild-type, 30°C, pH 7.0
0.06
ADP
mutant K48M, 30°C, pH 7.0
0.091
ADP
mutant R93M, 30°C, pH 7.0
0.093
ADP
mutant R111M, 30°C, pH 7.0
0.106
ADP
mutant K69M, 30°C, pH 7.0
0.118
ADP
mutant R73M, 30°C, pH 7.0
0.164
ADP
mutant R130M, 30°C, pH 7.0
0.238
ADP
mutant R84M, 30°C, pH 7.0
0.451
ADP
mutant R138M, 30°C, pH 7.0
5.62
ADP
mutant R141M, 30°C, pH 7.0
0.094
ATP
mutant R73M, 30°C, pH 7.0
0.101
ATP
mutant R93M, 30°C, pH 7.0
0.107
ATP
wild-type, 30°C, pH 7.0
0.229
ATP
mutant R130M, 30°C, pH 7.0
0.236
ATP
mutant K48M, 30°C, pH 7.0
0.268
ATP
mutant K69M, 30°C, pH 7.0
0.303
ATP
mutant R84M, 30°C, pH 7.0
0.518
ATP
mutant R138M, 30°C, pH 7.0
0.792
ATP
mutant R111M, 30°C, pH 7.0
5.2
ATP
mutant R141M, 30°C, pH 7.0
additional information
additional information
steady-state kinetics
-
additional information
additional information
-
steady-state kinetics
-
additional information
additional information
-
kinetics of recombinant wild-type and mutant enzymes, overview
-
additional information
additional information
kinetics of recombinant wild-type and mutant enzymes, overview
-
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D23N
site-directed mutagenesis, the mutant shows highly reduced activity in both forward and reverse reactions compared to the wild-type enzyme
K17M
site-directed mutagenesis, the mutant shows reduced activity in both forward and reverse reactions compared to the wild-type enzyme
K19M
site-directed mutagenesis, the mutant shows highly reduced activity in both forward and reverse reactions compared to the wild-type enzyme
K22M
site-directed mutagenesis, the mutant shows a 10000fold reduced activity in both forward and reverse reactions compared to the wild-type enzyme, almost inactive mutant
K48M
site-directed mutagenesis, the mutant shows altered kinetics and reduced activity in both forward and reverse reactions compared to the wild-type enzyme
R110M
site-directed mutagenesis, the mutant shows altered kinetics and highly reduced activity in both forward and reverse reactions compared to the wild-type enzyme
R111M
site-directed mutagenesis, the mutant shows altered kinetics and reduced activity in both forward and reverse reactions compared to the wild-type enzyme
R130M
site-directed mutagenesis, the mutant shows altered kinetics and reduced activity in both forward and reverse reactions compared to the wild-type enzyme
R138M
site-directed mutagenesis, the mutant shows altered kinetics and reduced activity in both forward and reverse reactions compared to the wild-type enzyme
R141M
site-directed mutagenesis, the mutant shows altered kinetics and reduced activity in both forward and reverse reactions compared to the wild-type enzyme
R18Q
site-directed mutagenesis, the mutant shows highly reduced activity in both forward and reverse reactions compared to the wild-type enzyme
R73M
site-directed mutagenesis, the mutant shows altered kinetics and reduced activity in both forward and reverse reactions compared to the wild-type enzyme
R84M
site-directed mutagenesis, the mutant shows altered kinetics and reduced activity in both forward and reverse reactions compared to the wild-type enzyme
R93M
site-directed mutagenesis, the mutant shows altered kinetics and reduced activity in both forward and reverse reactions compared to the wild-type enzyme
K48M
mutant exhibits diminished Vmax values in both reaction directions with only slight Km perturbations
K69M
mutant exhibits diminished Vmax values in both reaction directions with only slight Km perturbations
R110M
no catalytic activity
R111M
substantially inflated Km values for mevalonate 5-phosphate and mevalonate 5-diphosphate
R130M
mutant exhibits slight changes in Vmax values in both reaction directions with slight Km perturbations
R138M
mutant exhibits slight changes in Vmax values in both reaction directions with slight Km perturbations
R141M
50fold increase in Km value for ATP, 120fold increase for ADP
R73M
mutant exhibits diminished Vmax values in both reaction directions with only slight Km perturbations
R84M
50- and 33fold increase in Km value for mevalonate 5-phosphate and mevalonate 5-diphosphate, respectively
R93M
mutant exhibits slight changes in Vmax values in both reaction directions with slight Km perturbations
additional information
analysis of the genetic basis of disseminated superficial porokeratosis (DSP) in two five-generation Chinese families with members diagnosed with DSP, whole-exome sequencing and genotyping. Identification of a nonsense variation c.412C > T (p.Arg138*) in the phosphomevalonate kinase gene (PMVK), which encodes a cytoplasmic enzyme catalyzing the conversion of mevalonate 5-phosphate to mevalonate 5-diphosphate in the mevalonate pathway. This genetic variant is involved in the development of DSP in both families. Using HaCaT cells as models, it is revealed that this variant disturbs subcellular localization, expression, and solubility of PMVK, apparent apoptosis in and under the cornoid lamella of PMVK-deficient lesional tissues is observed, with incomplete differentiation of keratinocytes. The R138* mutant shows reduced expression and solubility. Phenotypes, overview
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Olivier, L.M.; Chambliss, K.L.; Gibson, K.M.; Krisans, S.K.
Characterization of phosphomevalonate kinase: chromosomal localization, regulation, and subcellular targeting
J. Lipid Res.
40
672-679
1999
Rattus norvegicus, Homo sapiens (Q15126), Homo sapiens
brenda
Hogenboom, S.; Tuyp, J.J.; Espeel, M.; Koster, J.; Wanders, R.J.; Waterham, H.R.
Phosphomevalonate kinase is a cytosolic protein in humans
J. Lipid Res.
45
697-705
2004
Homo sapiens
brenda
Herdendorf, T.J.; Miziorko, H.M.
Phosphomevalonate kinase: functional investigation of the recombinant human enzyme
Biochemistry
45
3235-3242
2006
Homo sapiens (Q15126), Homo sapiens
brenda
Herdendorf, T.J.; Miziorko, H.M.
Functional evaluation of conserved basic residues in human phosphomevalonate kinase
Biochemistry
46
11780-11788
2007
Homo sapiens, Homo sapiens (Q15126)
brenda
Olson, A.L.; Yao, H.; Herdendorf, T.J.; Miziorko, H.M.; Hannongbua, S.; Saparpakorn, P.; Cai, S.; Sem, D.S.
Substrate induced structural and dynamics changes in human phosphomevalonate kinase and implications for mechanism
Proteins
75
127-138
2008
Homo sapiens
brenda
Chang, Q.; Yan, X.; Gu, S.; Liu, J.; Liang, D.
Crystal structure of human phosphomevalonate kinase at 1.8 A resolution
Proteins Struct. Funct. Bioinform.
73
254-258
2008
Homo sapiens (Q15126)
brenda
Chang, Q.; Yan, X.; Gu, S.; Liu, J.; Liang, D.
Crystal structure of human phosphomevalonate kinase at 1.8 A resolution
Proteins
73
254-258
2008
Homo sapiens
brenda
Boonsri, P.; Neumann, T.S.; Olson, A.L.; Cai, S.; Herdendorf, T.J.; Miziorko, H.M.; Hannongbua, S.; Sem, D.S.
Molecular docking and NMR binding studies to identify novel inhibitors of human phosphomevalonate kinase
Biochem. Biophys. Res. Commun.
430
313-319
2013
Homo sapiens (Q15126), Homo sapiens
brenda
Nowroozi, F.F.; Baidoo, E.E.; Ermakov, S.; Redding-Johanson, A.M.; Batth, T.S.; Petzold, C.J.; Keasling, J.D.
Metabolic pathway optimization using ribosome binding site variants and combinatorial gene assembly
Appl. Microbiol. Biotechnol.
98
1567-1581
2014
Homo sapiens
brenda
Wang, J.; Liu, Y.; Liu, F.; Huang, C.; Han, S.; Lv, Y.; Liu, C.J.; Zhang, S.; Qin, Y.; Ling, L.; Gao, M.; Yu, S.; Li, C.; Huang, M.; Liao, S.; Hu, X.; Lu, Z.; Liu, X.; Jiang, T.; Tang, Z.; Zhang, H.; Guo, A.Y.; Liu, M.
Loss-of-function mutation in PMVK causes autosomal dominant disseminated superficial porokeratosis
Sci. Rep.
6
24226
2016
Homo sapiens (Q15126)
brenda