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Information on EC 3.1.3.43 - [pyruvate dehydrogenase (acetyl-transferring)]-phosphatase and Organism(s) Homo sapiens and UniProt Accession Q9P0J1

for references in articles please use BRENDA:EC3.1.3.43
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IUBMB Comments
A mitochondrial enzyme associated with EC 1.2.4.1 pyruvate dehydrogenase (acetyl-transferring), in the pyruvate dehydrogenase complex.
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This record set is specific for:
Homo sapiens
UNIPROT: Q9P0J1
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The taxonomic range for the selected organisms is: Homo sapiens
The expected taxonomic range for this enzyme is: Eukaryota, Bacteria
Synonyms
pyruvate dehydrogenase complex, pyruvate dehydrogenase phosphatase, pdh phosphatase, pyruvate dehydrogenase phosphate phosphatase, pyruvate dehydrogenase phosphatase 1, pda1p, pyruvate dehydrogenase phosphatase 2, yor090cp, phosphopyruvate dehydrogenase phosphatase, pyruvate dehydrogenase phosphatase isoform 1, more
SYNONYM
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
PDP1
isoform
pyruvate dehydrogenase phosphatase
-
pyruvate dehydrogenase phosphatase isoform 1
-
PDH phosphatase
-
-
phosphatase, pyruvate dehydrogenase
-
-
-
-
phosphopyruvate dehydrogenase phosphatase
-
-
-
-
pyruvate dehydrogenase phosphatase
pyruvate dehydrogenase phosphatase 1
-
-
pyruvate dehydrogenase phosphatase 2
-
-
pyruvate dehydrogenase phosphatase isoform 2
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pyruvate dehydrogenase phosphate phosphatase
-
-
-
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[pyruvate dehydrogenase (acetyl-transferring)]-phosphatase
-
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[pyruvate dehydrogenase (lipoamide)]-phosphatase
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-
-
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[pyruvate dehydrogenase (lipoamide)]-phosphate phosphohydrolase
-
-
-
-
REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
hydrolysis of phosphoric ester
SYSTEMATIC NAME
IUBMB Comments
[pyruvate dehydrogenase (acetyl-transferring)]-phosphate phosphohydrolase
A mitochondrial enzyme associated with EC 1.2.4.1 pyruvate dehydrogenase (acetyl-transferring), in the pyruvate dehydrogenase complex.
CAS REGISTRY NUMBER
COMMENTARY hide
9073-70-5
-
SUBSTRATE
PRODUCT                       
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
[pyruvate dehydrogenase (acetyl-transferring)] phosphate + H2O
[pyruvate dehydrogenase (acetyl-transferring)] + phosphate
show the reaction diagram
-
-
-
-
?
[pyruvate dehydrogenase (lipoamide)]-phosphate + H2O
?
show the reaction diagram
-
-
-
-
?
[pyruvate dehydrogenase (lipoamide)]-phosphate + H2O
[pyruvate dehydrogenase (lipoamide)] + phosphate
show the reaction diagram
additional information
?
-
-
dephosphorylation of substrate and substrate mutants R234Q, R234G occurs at similar rates at phosphorylation sites 1 and 2, the rate of dephosphorylation of site 3 is about 50% of wild type in substrate mutant R234Q
-
?
NATURAL SUBSTRATE
NATURAL PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
[pyruvate dehydrogenase (acetyl-transferring)] phosphate + H2O
[pyruvate dehydrogenase (acetyl-transferring)] + phosphate
show the reaction diagram
-
-
-
-
?
[pyruvate dehydrogenase (lipoamide)]-phosphate + H2O
?
show the reaction diagram
-
-
-
-
?
[pyruvate dehydrogenase (lipoamide)]-phosphate + H2O
[pyruvate dehydrogenase (lipoamide)] + phosphate
show the reaction diagram
-
-
-
-
?
METALS and IONS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
Mg2+
-
dependent
ORGANISM
COMMENTARY hide
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
-
UniProt
Manually annotated by BRENDA team
SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
SOURCE
-
PDP1 is downregulated in obese subjects
Manually annotated by BRENDA team
-
pyruvate dehydrogenase subunit PDHA1 and pyruvate dehydrogenase phosphatase PDP1 are frequently amplified and overexpressed at both the gene and protein levels in prostate tumors
Manually annotated by BRENDA team
-
patients with congenital lactic acidemia
Manually annotated by BRENDA team
GENERAL INFORMATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
metabolism
-
the enzyme is involved in the regulation of pyruvate dehydrogenase performing its activating dephosphorylation, together with phosphorylation and inactivation by pyruvate dehydrogenase kinase, PDK. Pyruvate dehydrogenase phosphatase PDP1 activity and expression is positively correlated with citrate synthase activity, regulation, overview
physiological function
UNIPROT
ENTRY NAME
ORGANISM
NO. OF AA
NO. OF TRANSM. HELICES
MOLECULAR WEIGHT[Da]
SOURCE
SEQUENCE
LOCALIZATION PREDICTION?
PDP1_HUMAN
537
0
61054
Swiss-Prot
other Location (Reliability: 4)
POSTTRANSLATIONAL MODIFICATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
phosphoprotein
CRYSTALLIZATION (Commentary)
ORGANISM
UNIPROT
LITERATURE
hanging-drop method
hanging-drop method
PROTEIN VARIANTS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
E93X
the nonsense mutation in the PDP1 gene shows a complete absence of isoform PDP1 protein in mitochondria, PDP2 can compensate for loss of PDP1 protein in a null patient
E93X
the nonsense mutation in the PDP1 gene shows a complete absence of isoform PDP1 protein in mitochondria, PDP2 can compensate for loss of PDP1 protein in a null patient
Y94F
-
phosphorylation-deficient mutant. Expression of the mutant in H-1299 cells leads to decreased proliferation under hypoxia and increased oxidative phosphorylation
PURIFICATION (Commentary)
ORGANISM
UNIPROT
LITERATURE
immobilized metal ion affinity chromatography (Ni2+), gel filtration
75% purity after Ni-affinity chromatography
-
immobilized metal ion affinity chromatography (Ni2+), gel filtration
CLONED (Commentary)
ORGANISM
UNIPROT
LITERATURE
His-tagged version expressed in Escherichia coli BL21
His-tag fusion protein, expression in Escherichia coli BL21(DE3)
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His-tagged SUMO version expressed in Escherichia coli BL21
EXPRESSION
ORGANISM
UNIPROT
LITERATURE
an oncogene BRAFV600E-induced senescence is accompanied by induction of the pyruvate dehydrogenase phosphatase 2
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gene expression is correlated to exercise-training
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APPLICATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
medicine
molecular biology
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pyruvate dehydrogenase phosphatase acts as a Smad1 phosphatase and plays a key role in TGF-b signalling pathways
REF.
AUTHORS
TITLE
JOURNAL
VOL.
PAGES
YEAR
ORGANISM (UNIPROT)
PUBMED ID
SOURCE
Ito, M.; Kobashi, H.; Naito, E.; Saijo, T.; Takeda, E.; Huq, A.H.M.M.; Kuroda, Y.
Decrease of pyruvate dehydrogenase phosphatase activity in patients with congenital lactic acidemia
Clin. Chim. Acta
209
1-7
1992
Homo sapiens
Manually annotated by BRENDA team
Jacobia, S.J.; Korotchkina, L.G.; Patel, M.S.
Differential effects of two mutations at arginine-234 in the alpha subunit of human pyruvate dehydrogenase
Arch. Biochem. Biophys.
395
121-128
2001
Homo sapiens
Manually annotated by BRENDA team
Chen, H.B.; Shen, J.; Ip, Y.T.; Xu, L.
Identification of phosphatases for Smad in the BMP/DPP pathway
Genes Dev.
20
648-653
2006
Drosophila melanogaster, Homo sapiens
Manually annotated by BRENDA team
Maj, M.C.; MacKay, N.; Levandovskiy, V.; Addis, J.; Baumgartner, E.R.; Baumgartner, M.R.; Robinson, B.H.; Cameron, J.M.
Pyruvate dehydrogenase phosphatase deficiency: identification of the first mutation in two brothers and restoration of activity by protein complementation
J. Clin. Endocrinol. Metab.
90
4101-4107
2005
Homo sapiens
Manually annotated by BRENDA team
Piccinini, M.; Mostert, M.; Alberto, G.; Ramondetti, C.; Novi, R.F.; Dalmasso, P.; Rinaudo, M.T.
Down-regulation of pyruvate dehydrogenase phosphatase in obese subjects is a defect that signals insulin resistance
Obes. Res.
13
678-686
2005
Homo sapiens
Manually annotated by BRENDA team
Cameron, J.M.; Maj, M.; Levandovskiy, V.; Barnett, C.P.; Blaser, S.; Mackay, N.; Raiman, J.; Feigenbaum, A.; Schulze, A.; Robinson, B.H.
Pyruvate dehydrogenase phosphatase 1 (PDP1) null mutation produces a lethal infantile phenotype
Hum. Genet.
125
319-326
2009
Homo sapiens (Q9P0J1), Homo sapiens (Q9P2J9), Homo sapiens
Manually annotated by BRENDA team
Kato, J.; Kato, M.
Crystallization and preliminary crystallographic studies of the catalytic subunits of human pyruvate dehydrogenase phosphatase isoforms 1 and 2
Acta Crystallogr. Sect. F
66
342-345
2010
Homo sapiens (Q9P0J1), Homo sapiens (Q9P2J9), Homo sapiens
Manually annotated by BRENDA team
Love, L.; LeBlanc, P.; Inglis, J.; Bradley, N.; Choptiany, J.; Heigenhauser, G.; Peters, S.
The relationship between human skeletal muscle pyruvate dehydrogenase phosphatase activity and muscle aerobic capacity
J. Appl. Physiol.
111
427-434
2011
Homo sapiens
Manually annotated by BRENDA team
Shan, C.; Kang, H.B.; Elf, S.; Xie, J.; Gu, T.L.; Aguiar, M.; Lonning, S.; Hitosugi, T.; Chung, T.W.; Arellano, M.; Khoury, H.J.; Shin, D.M.; Khuri, F.R.; Boggon, T.J.; Fan, J.
Tyr-94 phosphorylation inhibits pyruvate dehydrogenase phosphatase 1 and promotes tumor growth
J. Biol. Chem.
289
21413-21422
2014
Homo sapiens
Manually annotated by BRENDA team
Kaplon, J.; Zheng, L.; Meissl, K.; Chaneton, B.; Selivanov, V.A.; Mackay, G.; van der Burg, S.H.; Verdegaal, E.M.; Cascante, M.; Shlomi, T.; Gottlieb, E.; Peeper, D.S.
A key role for mitochondrial gatekeeper pyruvate dehydrogenase in oncogene-induced senescence
Nature
498
109-112
2013
Homo sapiens
Manually annotated by BRENDA team
Wang, H.; Zhang, L.; Guo, X.; Bai, Y.; Li, Y.X.; Sha, J.; Peng, C.; Wang, Y.L.; Liu, M.
MiR-195 modulates oxidative stress-induced apoptosis and mitochondrial energy production in human trophoblasts via flavin adenine dinucleotide-dependent oxidoreductase domain-containing protein 1 and pyruvate dehydrogenase phosphatase regulatory subunit
J. Hypertens.
36
306-318
2018
Homo sapiens (Q8NCN5), Homo sapiens
Manually annotated by BRENDA team
Barberio, M.D.; Huffman, K.M.; Giri, M.; Hoffman, E.P.; Kraus, W.E.; Hubal, M.J.
Pyruvate dehydrogenase phosphatase regulatory gene expression correlates with exercise training insulin sensitivity changes
Med. Sci. Sports Exerc.
48
2387-2397
2016
Homo sapiens
Manually annotated by BRENDA team
Chen, J.; Guccini, I.; Mitri, D.; Brina, D.; Revandkar, A.; Sarti, M.; Pasquini, E.; Alajati, A.; Pinton, S.; Losa, M.; Civenni, G.; Catapano, C.; Sgrignani, J.; Cavalli, A.; DAntuono, R.; Asara, J.; Morandi, A.; Chiarugi, P.; Crotti, S.; Agostini, M.
Compartmentalized activities of the pyruvate dehydrogenase complex sustain lipogenesis in prostate cancer
Nat. Genet.
50
219-228
2018
Homo sapiens
Manually annotated by BRENDA team
Kono, M.; Yoshida, N.; Maeda, K.; Skinner, N.E.; Pan, W.; Kyttaris, V.C.; Tsokos, M.G.; Tsokos, G.C.
Pyruvate dehydrogenase phosphatase catalytic subunit 2 limits Th17 differentiation
Proc. Natl. Acad. Sci. USA
115
9288-9293
2018
Homo sapiens, Mus musculus
Manually annotated by BRENDA team