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Showing PDP1 — PDP is a alias.

PDP1

Polyisoprenoid diphosphate/phosphate phosphohydrolase PLPP6 · UniProt Q8IY26

Length
295 aa
Mass
32.2 kDa
Annotated
2026-06-10
100 papers in source corpus 10 papers cited in narrative 10 extracted findings
Cross-family judge vs UniProt: Affinage preferred faithfulness: 8/8 claims corpus-supported (100%)

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

PDP1 is a mitochondrial phosphatase that dephosphorylates and activates the pyruvate dehydrogenase complex (PDC), serving as a key node that couples glucose oxidation to cellular energy demand (PMID:19184109, PMID:33725483). A null mutation that abolishes PDP1 protein eliminates PDC activation, and native activity is restored by recombinant PDP1 (with PDP2 acting as a compensatory isoform), establishing PDP1's direct catalytic role on the PDHA1 (E1α) subunit (PMID:19184109). PDP1 activity is governed by hierarchical post-translational modifications: ACAT1-mediated K202 acetylation inhibits PDP1 by dissociating its PDHA1 substrate, SIRT3-mediated deacetylation reverses this, and Y381 phosphorylation toggles the recruitment of ACAT1 versus SIRT3 to the complex (PMID:24486017). Upstream regulators tune this axis in distinct physiological contexts: Rheb physically associates with PDP1 to enhance its activity and PDH dephosphorylation in response to synaptic activity (PMID:33725483), while FLT3-ITD/RAS signaling induces PDP1 expression to sustain oxidative glucose metabolism, respiratory capacity, and proliferation in AML (PMID:37935978). Beyond mitochondrial catabolism, PDP1-dependent PDH activation supplies acetyl-CoA that maintains histone H3 acetylation, sustaining HIF-1 transcriptional activity at hypoxia-response elements (PMID:36453802) and modulating histone acetylation at DNA damage repair sites (PMID:34489398). PDP1 additionally functions non-enzymatically as a scaffold that enhances BRAF–MEK1 interaction and MAPK signaling in KRAS-mutant colorectal cancer (PMID:38849010). Biallelic loss-of-function mutations in PDP1 cause pyruvate dehydrogenase complex deficiency (PMID:19184109, PMID:31392110). Note that the alias PPAPDC2, an ER/nuclear-envelope polyisoprenoid diphosphate phosphatase sharing the HGNC symbol, is a distinct protein (PMID:20110354).

Mechanistic history

Synthesis pass · year-by-year structured walk · 9 steps
  1. 2009 High

    Established that PDP1 is directly required for PDC activation in human cells, resolving whether its phosphatase activity is essential versus redundant.

    Evidence Identification of a homozygous nonsense mutation abolishing PDP1, with enzymatic reconstitution by recombinant PDP1/PDP2 in patient fibroblasts

    PMID:19184109

    Open questions at the time
    • Did not define the post-translational regulation of PDP1 activity
    • Degree and physiological scope of PDP2 compensation unclear
  2. 2010 Medium

    Characterized PPAPDC2 (an alias sharing the PDP1/HGNC symbol) as a distinct ER/nuclear-envelope polyisoprenoid diphosphate phosphatase, flagging a symbol collision.

    Evidence Recombinant enzyme in vitro substrate assays, yeast sterol auxotrophy, and isoprenylation assays in mammalian cells

    PMID:20110354

    Open questions at the time
    • This is a different protein from pyruvate dehydrogenase phosphatase PDP1
    • Endogenous physiological role of PPAPDC2 not established
  3. 2014 High

    Defined the hierarchical PTM logic controlling PDP1, showing how acetylation and tyrosine phosphorylation gate writer/eraser recruitment to the PDC.

    Evidence In vitro acetylation/deacetylation assays, Co-IP, site-directed mutagenesis (K202, Y381), and mass spectrometry

    PMID:24486017

    Open questions at the time
    • Upstream kinase responsible for Y381 phosphorylation not identified
    • Physiological signals driving ACAT1/SIRT3 switching not mapped
  4. 2021 High

    Identified Rheb as a direct upstream activator linking neuronal/synaptic activity to PDP1-driven PDH activation and ATP production.

    Evidence Co-IP, cell-type-specific gain- and loss-of-function genetic models, PDH phosphorylation/activity and acetyl-CoA/ATP measurements

    PMID:33725483

    Open questions at the time
    • Structural basis of Rheb-PDP1 association unknown
    • Whether Rheb effect is GTP-dependent not resolved here
  5. 2021 Medium

    Connected PDP1 to the nucleus by showing the [Ca2+]m-PDP1-PDH axis controls histone acetylation at DNA damage repair sites and radioresistance.

    Evidence Western blotting, NDUFS1 gain-of-function rescue, nuclear fractionation, histone acetylation assays, and in vivo tumor models

    PMID:34489398

    Open questions at the time
    • Mechanism of nuclear PDH/PDP1 trafficking not detailed
    • Single lab; direct PDP1 contribution versus complex-level effect not separated
  6. 2022 High

    Extended PDP1's metabolic role to epigenetic control of transcription, showing acetyl-CoA supply sustains HIF-1 target gene expression independent of HIF-1α stability.

    Evidence siRNA knockdown, ChIP, luciferase reporters, and acetate/HDAC-inhibitor rescue experiments

    PMID:36453802

    Open questions at the time
    • Acetyltransferase(s) acting on H3 at HIF-1 promoters not identified
    • Generality across cell types beyond those tested unclear
  7. 2022 Medium

    Placed PDP1 under microRNA control, showing miR-18a-3p directly represses PDP1 with downstream effects on chondrocyte inflammatory responses.

    Evidence Luciferase reporter binding validation, RT-qPCR, ELISA, and an in vivo rat OA model

    PMID:35148687

    Open questions at the time
    • Mechanistic link between PDP1 level and cytokine/MMP output not fully resolved
    • Single lab
  8. 2023 High

    Demonstrated oncogenic dependency: FLT3-ITD/RAS signaling induces PDP1 to maintain oxidative metabolism and proliferation in AML.

    Evidence Genome-wide CRISPR screens, NMR metabolic profiling, siRNA knockdown, respiratory measurements, and in vivo patient-derived blast experiments

    PMID:37935978

    Open questions at the time
    • Direct transcriptional effectors downstream of RAS driving PDP1 not pinpointed
    • Therapeutic window of PDP1 inhibition in AML untested
  9. 2023 Medium

    Revealed a moonlighting, phosphatase-independent function of PDP1 as a scaffold promoting BRAF-MEK1 interaction and MAPK signaling in KRAS-mutant cancer.

    Evidence Co-IP, CRISPR screens, endogenous protein tagging, in vitro and in vivo functional assays, and identification of KLF5 as a transcriptional driver

    PMID:38849010

    Open questions at the time
    • Structural basis of the scaffold interaction unresolved
    • How a mitochondrial enzyme accesses cytosolic BRAF-MEK1 not explained
    • Single lab

Open questions

Synthesis pass · forward-looking unresolved questions
  • How PDP1's distinct activities—mitochondrial phosphatase, acetyl-CoA-dependent epigenetic regulator, and cytosolic MAPK scaffold—are spatially and physiologically partitioned within a single cell remains unresolved.
  • No unified model reconciling mitochondrial, nuclear, and cytosolic pools
  • Kinase for Y381 and signals controlling localization unknown
  • No structural model of PDP1 in any of its complexes

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0140096 catalytic activity, acting on a protein 3 GO:0016787 hydrolase activity 2 GO:0060090 molecular adaptor activity 1
Localization
GO:0005634 nucleus 2 GO:0005739 mitochondrion 1
Pathway
R-HSA-1430728 Metabolism 3 R-HSA-162582 Signal Transduction 2 R-HSA-74160 Gene expression (Transcription) 2
Partners
ACAT1BRAFMAP2K1PDHA1RHEBSIRT3
Complex memberships
pyruvate dehydrogenase complex (PDC)

Evidence

Reading pass · 10 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
2014 K202 acetylation of PDP1 by mitochondrial acetyltransferase ACAT1 inhibits PDP1 activity by dissociating its substrate PDHA1, while SIRT3-mediated deacetylation reverses this inhibition. Additionally, Y381 phosphorylation of PDP1 dissociates SIRT3 from the pyruvate dehydrogenase complex (PDC) and recruits ACAT1, creating a hierarchical post-translational regulatory mechanism that controls PDC activity. In vitro acetylation/deacetylation assays, co-immunoprecipitation, site-directed mutagenesis, mass spectrometry, knockdown experiments with defined biochemical readouts Molecular cell High 24486017
2009 A null mutation in PDP1 (homozygous c.277G>T, p.E93X nonsense mutation) completely abolishes PDP1 protein in mitochondria and causes failure to activate the PDH complex; native PDHc activity could be restored by addition of recombinant PDP1 or PDP2, demonstrating PDP1's direct phosphatase role in PDHc activation and identifying PDP2 as a compensatory isoform. Immunoblotting, enzymatic reconstitution with recombinant PDP1/PDP2, fibroblast biochemical assays, genetic mutation identification Human genetics High 19184109
2021 Rheb physically associates with PDP1 (PDH phosphatase), enhancing PDP1 activity and its association with the catalytic E1α-subunit of PDH, thereby reducing PDH phosphorylation and increasing PDH activity to support neuronal ATP production in response to synaptic activity. Co-immunoprecipitation, cell-type-specific gain- and loss-of-function genetic models, PDH phosphorylation/activity assays, acetyl-CoA and ATP measurements Developmental cell High 33725483
2022 PDP1 supports HIF-1 transcriptional activity under hypoxia by maintaining acetyl-CoA production through PDH activation, which sustains histone H3 acetylation at HIF-1 target gene promoters and enables HIF-1 binding to hypoxia-response elements; PDP1 depletion reduces histone acetylation and HIF-1 target gene expression without affecting HIF-1α protein levels or nuclear accumulation. siRNA knockdown, chromatin immunoprecipitation (ChIP), luciferase reporter assays, acetate/HDAC inhibitor rescue experiments, HIF-1α interaction assays The FEBS journal High 36453802
2023 PDP1 acts as a scaffold protein that enhances BRAF and MEK1 interaction and activates MAPK signaling in KRAS-mutant colorectal cancer cells, promoting tumor progression through a mechanism distinct from its phosphatase activity on PDH. Transcription factor KLF5 drives PDP1 upregulation in KRAS-mutant CRC. Co-immunoprecipitation, in vitro and in vivo functional experiments, CRISPR screens, endogenous protein tagging, knockdown/overexpression studies Cancer letters Medium 38849010
2023 FLT3-ITD induces PDP1 expression through the RAS signaling axis in acute myeloid leukemia cells, and PDP1 functions as an activator of the pyruvate dehydrogenase complex to enhance oxidative glucose metabolism and maintain respiratory capacity; PDP1 knockdown reduces cellular respiration and impairs proliferation specifically in FLT3-ITD-positive cells. NMR metabolic profiling, genome-wide CRISPR screens, endogenous protein tagging, siRNA knockdown, respiratory capacity measurements, in vivo patient-derived blast experiments Leukemia High 37935978
2021 Mitochondrial complex I defects decrease PDP1 activity through the [Ca2+]m-PDP1-PDH axis, reducing nuclear PDH and consequently decreasing histone acetylation at DNA damage repair sites, promoting radioresistance in colorectal cancer cells. Western blotting, NDUFS1 overexpression, in vivo tumor models, nuclear fractionation, histone acetylation assays Cell death & disease Medium 34489398
2010 PDP1/PPAPDC2 (a distinct gene encoding an integral membrane lipid phosphatase, PPAPDC2) preferentially hydrolyzes polyisoprenoid diphosphates (FPP, GGPP) in vitro; localizes to the endoplasmic reticulum and nuclear envelope in mammalian cells; overexpression depletes FPP pools in yeast causing sterol auxotrophy and decreases protein isoprenylation in mammalian cells, causing defects in cell growth and cytoskeletal organization via dysregulation of Rho family GTPases. Tandem mass spectrometry assays, recombinant enzyme in vitro assays, yeast overexpression growth/sterol assays, subcellular fractionation/localization, isoprenylation assays in mammalian cells The Journal of biological chemistry Medium 20110354
2019 A novel biallelic frameshift mutation (c.575dupT, p.L192FfsX5) in PDP1 causes absent PDP1 protein and functional pyruvate dehydrogenase complex deficiency, with low PDC activities in lymphocytes and fibroblasts; unexpectedly, BCKDH activity was also reduced in fibroblasts, suggesting a potential shared regulatory function. Genetic sequencing, immunoblotting, enzymatic activity assays in fibroblasts and lymphocytes JIMD reports Medium 31392110
2022 miR-18a-3p directly targets PDP1 mRNA (validated by luciferase reporter assay) and negatively regulates PDP1 expression; PDP1 upregulation reverses the suppressive effect of miR-18a-3p on inflammatory cytokine secretion and matrix metalloproteinase levels in IL-1β-stimulated chondrocytes. Luciferase reporter assay, RT-qPCR, ELISA, western blotting, in vivo rat OA model The journal of physiological sciences : JPS Medium 35148687

Source papers

Stage 0 corpus · 100 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
2003 vrille, Pdp1, and dClock form a second feedback loop in the Drosophila circadian clock. Cell 426 12581523
2014 Tyr phosphorylation of PDP1 toggles recruitment between ACAT1 and SIRT3 to regulate the pyruvate dehydrogenase complex. Molecular cell 255 24486017
1998 Regulation of skeletal muscle glycogen phosphorylase and PDH at varying exercise power outputs. The American journal of physiology 144 9688676
1999 PUFA and aging modulate cardiac mitochondrial membrane lipid composition and Ca2+ activation of PDH. The American journal of physiology 131 9887028
2001 Human skeletal muscle PDH kinase activity and isoform expression during a 3-day high-fat/low-carbohydrate diet. American journal of physiology. Endocrinology and metabolism 128 11701428
2003 PDP: protein domain parser. Bioinformatics (Oxford, England) 117 12584135
2022 Smart PdH@MnO2 Yolk-Shell Nanostructures for Spatiotemporally Synchronous Targeted Hydrogen Delivery and Oxygen-Elevated Phototherapy of Melanoma. ACS nano 99 35315637
2020 Selective Transfer Semihydrogenation of Alkynes with H2 O (D2 O) as the H (D) Source over a Pd-P Cathode. Angewandte Chemie (International ed. in English) 84 32777146
2021 DCA Protects against Oxidation Injury Attributed to Cerebral Ischemia-Reperfusion by Regulating Glycolysis through PDK2-PDH-Nrf2 Axis. Oxidative medicine and cellular longevity 77 34712383
2021 Melatonin inhibits lung cancer development by reversing the Warburg effect via stimulating the SIRT3/PDH axis. Journal of pineal research 69 34214200
1990 Comparative epitope mapping of murine monoclonal and human autoantibodies to human PDH-E2, the major mitochondrial autoantigen of primary biliary cirrhosis. Journal of immunology (Baltimore, Md. : 1950) 69 1690773
2001 Muscle fiber type comparison of PDH kinase activity and isoform expression in fed and fasted rats. American journal of physiology. Regulatory, integrative and comparative physiology 68 11171643
1996 Dra-nupC-pdp operon of Bacillus subtilis: nucleotide sequence, induction by deoxyribonucleosides, and transcriptional regulation by the deoR-encoded DeoR repressor protein. Journal of bacteriology 65 8550462
2016 Metformin increases PDH and suppresses HIF-1α under hypoxic conditions and induces cell death in oral squamous cell carcinoma. Oncotarget 64 27474170
2005 PDH kinase inhibitors: a novel therapy for Type II diabetes? Biochemical Society transactions 64 15787608
2015 The PI3K/Akt Pathway Regulates Oxygen Metabolism via Pyruvate Dehydrogenase (PDH)-E1α Phosphorylation. Molecular cancer therapeutics 60 25995437
2011 PDP-1 links the TGF-β and IIS pathways to regulate longevity, development, and metabolism. PLoS genetics 59 21533078
2017 Improving acetyl-CoA biosynthesis in Saccharomyces cerevisiae via the overexpression of pantothenate kinase and PDH bypass. Biotechnology for biofuels 58 28239413
2018 Poldip2 is an oxygen-sensitive protein that controls PDH and αKGDH lipoylation and activation to support metabolic adaptation in hypoxia and cancer. Proceedings of the National Academy of Sciences of the United States of America 56 29434038
2004 Simulation of Drosophila circadian oscillations, mutations, and light responses by a model with VRI, PDP-1, and CLK. Biophysical journal 54 15111397
2018 Pyruvate Dehydrogenase PDH-E1β Controls Tumor Progression by Altering the Metabolic Status of Cancer Cells. Cancer research 52 29436427
1988 Localization of pigment-dispersing hormone (PDH) immunoreactivity in the central nervous system of Carcinus maenas and Orconectes limosus (Crustacea), with reference to FMRFamide immunoreactivity in O. limosus. Cell and tissue research 48 3416337
2021 Vitamin C activates pyruvate dehydrogenase (PDH) targeting the mitochondrial tricarboxylic acid (TCA) cycle in hypoxic KRAS mutant colon cancer. Theranostics 47 33664850
2006 Novel mutations in dihydrolipoamide dehydrogenase deficiency in two cousins with borderline-normal PDH complex activity. American journal of medical genetics. Part A 47 16770810
2004 Pigment-dispersing hormone (PDH)-immunoreactive neurons form a direct coupling pathway between the bilaterally symmetric circadian pacemakers of the cockroach Leucophaea maderae. Cell and tissue research 47 15578273
2011 Beneficial effect of pyruvate therapy on Leigh syndrome due to a novel mutation in PDH E1α gene. Brain & development 46 21454027
2021 Rheb mediates neuronal-activity-induced mitochondrial energetics through mTORC1-independent PDH activation. Developmental cell 44 33725483
2019 Lactic acidosis caused by repressed lactate dehydrogenase subunit B expression down-regulates mitochondrial oxidative phosphorylation via the pyruvate dehydrogenase (PDH)-PDH kinase axis. The Journal of biological chemistry 44 30923124
2012 Solution structure of the Pdp1 PWWP domain reveals its unique binding sites for methylated H4K20 and DNA. The Biochemical journal 44 22150589
2009 An isoform-specific mutant reveals a role of PDP1 epsilon in the circadian oscillator. The Journal of neuroscience : the official journal of the Society for Neuroscience 44 19726650
1997 Neurites of period-expressing PDH cells in the fly's optic lobe exhibit circadian oscillations in morphology. The European journal of neuroscience 42 9283834
1997 PDP1, a novel Drosophila PAR domain bZIP transcription factor expressed in developing mesoderm, endoderm and ectoderm, is a transcriptional regulator of somatic muscle genes. Development (Cambridge, England) 42 9409684
2021 Mitochondrial dysfunction induces radioresistance in colorectal cancer by activating [Ca2+]m-PDP1-PDH-histone acetylation retrograde signaling. Cell death & disease 41 34489398
2000 Pyruvate overrides inhibition of PDH during exercise after a low-carbohydrate diet. American journal of physiology. Endocrinology and metabolism 39 10913026
2000 The Drosophila PAR domain protein 1 (Pdp1) gene encodes multiple differentially expressed mRNAs and proteins through the use of multiple enhancers and promoters. Developmental biology 39 10926776
1991 Differential distribution of beta-pigment-dispersing hormone (beta-PDH)-like immunoreactivity in the stomatogastric nervous system of five species of decapod crustaceans. Cell and tissue research 37 1913777
2010 Circadian clock regulates response to pesticides in Drosophila via conserved Pdp1 pathway. Toxicological sciences : an official journal of the Society of Toxicology 35 20348229
2016 Age-related impairments in skeletal muscle PDH phosphorylation and plasma lactate are indicative of metabolic inflexibility and the effects of exercise training. American journal of physiology. Endocrinology and metabolism 34 27221120
1999 Identification and characterization of a DeoR-specific operator sequence essential for induction of dra-nupC-pdp operon expression in Bacillus subtilis. Journal of bacteriology 34 10074062
2019 pdh modulate virulence through reducing stress tolerance and biofilm formation of Streptococcus suis serotype 2. Virulence 33 31232165
2017 Regulation of pyruvate dehydrogenase (PDH) in the hibernating ground squirrel, (Ictidomys tridecemlineatus). Journal of thermal biology 33 29037383
2007 Regulation of PDH in human arm and leg muscles at rest and during intense exercise. American journal of physiology. Endocrinology and metabolism 31 17957032
2008 Cloning and differential expression of two beta-pigment-dispersing hormone (beta-PDH) isoforms in the crab Cancer productus: evidence for authentic beta-PDH as a local neurotransmitter and beta-PDH II as a humoral factor. The Journal of comparative neurology 30 18311785
2014 Prolyl-hydroxylase PHD3 interacts with pyruvate dehydrogenase (PDH)-E1β and regulates the cellular PDH activity. Biochemical and biophysical research communications 29 25088999
1998 TNF binding protein prevents hyperlactatemia and inactivation of PDH complex in skeletal muscle during sepsis. The Journal of surgical research 29 9790813
1996 Fasting and decreased B cell sensitivity: important role for fatty acid-induced inhibition of PDH activity. The American journal of physiology 29 8764183
2010 Functional characterization of the atypical integral membrane lipid phosphatase PDP1/PPAPDC2 identifies a pathway for interconversion of isoprenols and isoprenoid phosphates in mammalian cells. The Journal of biological chemistry 28 20110354
2019 Structural and Functional Analyses of the Human PDH Complex Suggest a "Division-of-Labor" Mechanism by Local E1 and E3 Clusters. Structure (London, England : 1993) 27 31130485
2009 Low muscle glycogen and elevated plasma free fatty acid modify but do not prevent exercise-induced PDH activation in human skeletal muscle. Diabetes 27 19833896
1986 Phytohemagglutinin (PHA) activated human T-lymphocytes: concomitant appearance of insulin binding, degradation and insulin-mediated activation of pyruvate dehydrogenase (PDH). Biochemical and biophysical research communications 27 3511901
2003 Regulation of PDH activity and isoform expression: diet and exercise. Biochemical Society transactions 26 14641042
1990 Antibody binding and inhibition of pyruvate dehydrogenase (PDH) in sera from patients with primary biliary cirrhosis. Clinical and experimental immunology 26 2387091
2017 Metschnikowia pulcherrima Influences the Expression of Genes Involved in PDH Bypass and Glyceropyruvic Fermentation in Saccharomyces cerevisiae. Frontiers in microbiology 25 28702001
2010 PGC-1alpha increases PDH content but does not change acute PDH regulation in mouse skeletal muscle. American journal of physiology. Regulatory, integrative and comparative physiology 25 20720174
1993 Characterization of PDH beta 1, the structural gene for the pyruvate dehydrogenase beta subunit from Saccharomyces cerevisiae. Proceedings of the National Academy of Sciences of the United States of America 25 8433986
2017 PGC-1α and fasting-induced PDH regulation in mouse skeletal muscle. Physiological reports 24 28400503
2009 Pyruvate dehydrogenase phosphatase 1 (PDP1) null mutation produces a lethal infantile phenotype. Human genetics 24 19184109
1993 Structure and localization of mRNA encoding a pigment dispersing hormone (PDH) in the eyestalk of the crayfish Orconectes limosus. FEBS letters 24 8477858
1991 Fraction of hepatic cytosolic acetyl-CoA derived from glucose in vivo: relation to PDH phosphorylation state. The American journal of physiology 24 2058663
2019 PDH-mediated metabolic flow is critical for skeletal muscle stem cell differentiation and myotube formation during regeneration in mice. FASEB journal : official publication of the Federation of American Societies for Experimental Biology 23 30939245
2012 Protein domain histochemistry (PDH): binding of the carbohydrate recognition domain (CRD) of recombinant human glycoreceptor CLEC10A (CD301) to formalin-fixed, paraffin-embedded breast cancer tissues. The journal of histochemistry and cytochemistry : official journal of the Histochemistry Society 23 23275449
2024 Baicalin attenuates neuronal damage associated with SDH activation and PDK2-PDH axis dysfunction in early reperfusion. Phytomedicine : international journal of phytotherapy and phytopharmacology 22 38579645
2023 Enabling growth-decoupled Komagataella phaffii recombinant protein production based on the methanol-free PDH promoter. Frontiers in bioengineering and biotechnology 22 37034257
2011 PDH activation during in vitro muscle contractions in PDH kinase 2 knockout mice: effect of PDH kinase 1 compensation. American journal of physiology. Regulatory, integrative and comparative physiology 22 21411764
1992 Pyruvate dehydrogenase (PDH) deficiency caused by a 21-base pair insertion mutation in the E1 alpha subunit. Human genetics 21 1551669
2022 Pyruvate dehydrogenase phosphatase 1 (PDP1) stimulates HIF activity by supporting histone acetylation under hypoxia. The FEBS journal 20 36453802
2010 The pdh operon is expressed in a subpopulation of stationary-phase bacteria and is important for survival of sugar-starved Streptococcus mutans. Journal of bacteriology 20 20581205
2009 PDH E1β deficiency with novel mutations in two patients with Leigh syndrome. Journal of inherited metabolic disease 20 19924563
2005 Elevated n-3 fatty acids in a high-fat diet attenuate the increase in PDH kinase activity but not PDH activity in human skeletal muscle. Journal of applied physiology (Bethesda, Md. : 1985) 20 15591305
2002 Glucose-stimulated insulin secretion does not require activation of pyruvate dehydrogenase: impact of adenovirus-mediated overexpression of PDH kinase and PDH phosphate phosphatase in pancreatic islets. Biochemical and biophysical research communications 20 11866475
1991 Pyruvate dehydrogenase deficiency due to a 20-bp deletion in exon II of the pyruvate dehydrogenase (PDH) E1 alpha gene. American journal of human genetics 20 1907799
2014 Two beta-pigment-dispersing hormone (β-PDH) isoforms in the mud crab, Scylla paramamosain: implication for regulation of ovarian maturation and a photoperiod-related daily rhythmicity. Animal reproduction science 19 25262380
2012 Cafestol to Tricalysiolide B and Oxidized Analogues: Biosynthetic and Derivatization Studies Using Non-heme Iron Catalyst Fe(PDP). Synlett : accounts and rapid communications in synthetic organic chemistry 19 23585710
2000 Catabolite repression of dra-nupC-pdp operon expression in Bacillus subtilis. Microbiology (Reading, England) 19 11065368
2022 Abnormal lipid droplets accumulation induced cognitive deficits in obstructive sleep apnea syndrome mice via JNK/SREBP/ACC pathway but not through PDP1/PDC pathway. Molecular medicine (Cambridge, Mass.) 18 35030992
2010 An animal model of PDH deficiency using AAV8-siRNA vector-mediated knockdown of pyruvate dehydrogenase E1α. Molecular genetics and metabolism 18 20685142
2006 Modelling of circadian rhythms in Drosophila incorporating the interlocked PER/TIM and VRI/PDP1 feedback loops. Journal of theoretical biology 17 17157878
2023 PDP1 is a key metabolic gatekeeper and modulator of drug resistance in FLT3-ITD-positive acute myeloid leukemia. Leukemia 16 37935978
2011 Role of pyruvate dehydrogenase kinase 4 in regulating PDH activation during acute muscle contraction. Applied physiology, nutrition, and metabolism = Physiologie appliquee, nutrition et metabolisme 16 22196220
2023 Loss of muscle PDH induces lactic acidosis and adaptive anaplerotic compensation via pyruvate-alanine cycling and glutaminolysis. The Journal of biological chemistry 15 37865313
2019 Metabolic reorganization in winter: Regulation of pyruvate dehydrogenase (PDH) during long-term freezing and anoxia. Cryobiology 15 30639451
2010 Precursor structure, distribution and possible functions of pigment-dispersing hormone (PDH) in the terrestrial isopod Armadillidium vulgare (Latreille). Journal of insect physiology 15 20637211
2005 The Drosophila Par domain protein I gene, Pdp1, is a regulator of larval growth, mitosis and endoreplication. Developmental biology 15 16313897
2023 PdP2 Nanoparticles on Reduced Graphene Oxide: A Catalyst for the Electrocatalytic Reduction of Nitrate to Ammonia. Inorganic chemistry 14 37074289
2022 MiR-18a-3p improves cartilage matrix remodeling and inhibits inflammation in osteoarthritis by suppressing PDP1. The journal of physiological sciences : JPS 14 35148687
2020 Porcine Digestible Peptides (PDP) in Weanling Diets Regulates the Expression of Genes Involved in Gut Barrier Function, Immune Response and Nutrient Transport in Nursery Pigs. Animals : an open access journal from MDPI 14 33321976
1997 Antibodies to P450IID6, SLA, PDH-E2 and BCKD-E2 in Japanese patients with chronic hepatitis. Journal of gastroenterology and hepatology 14 9504898
1995 Association of cerebral dysgenesis and lactic acidemia with X-linked PDH E1 alpha subunit mutations in females. Pediatric neurology 14 8771169
1999 Characterization of cryptic plasmids pDP1 and pSMB1 of Streptococcus pneumoniae. Plasmid 13 9887308
1996 Evolution of mammalian X-linked and autosomal Pgk and Pdh E1 alpha subunit genes. Molecular biology and evolution 13 8752009
1988 Retention of soman in rats, guinea-pigs and marmosets: species-dependent effects of the soman simulator, pinacolyl dimethylphosphinate (PDP). The Journal of pharmacy and pharmacology 13 2896771
2024 PDP1 promotes KRAS mutant colorectal cancer progression by serving as a scaffold for BRAF and MEK1. Cancer letters 12 38849010
2021 LDH and PDH Activities in the Ischemic Brain and the Effect of Reperfusion-An Ex Vivo MR Study in Rat Brain Slices Using Hyperpolarized [1-13C]Pyruvate. Metabolites 12 33808434
2021 Application Route and Immune Status of the Host Determine Safety and Oncolytic Activity of Oncolytic Coxsackievirus B3 Variant PD-H. Viruses 12 34696348
2021 Evaluation of immune effect of Streptococcus suis biofilm-associated protein PDH. Veterinary microbiology 12 34749282
2019 A novel null mutation in the pyruvate dehydrogenase phosphatase catalytic subunit gene (PDP1) causing pyruvate dehydrogenase complex deficiency. JIMD reports 12 31392110
2017 Induction of the PDH bypass and upregulation of the ALDH7B4 in plants treated with herbicides inhibiting amino acid biosynthesis. Plant science : an international journal of experimental plant biology 12 28969796
2001 A case of PDH-E1 alpha mosaicism in a male patient with severe metabolic lactic acidosis. Journal of inherited metabolic disease 12 11757583
2000 Sequential deletion of C-terminal amino acids of the E(1)alpha component of the pyruvate dehydrogenase (PDH) complex leads to reduced steady-state levels of functional E(1)alpha(2)beta(2) tetramers: implications for patients with PDH deficiency. Human molecular genetics 12 10767328
1991 Primary structure and relative potency of an analog of beta-PDH (pigment-dispersing hormone) from the crayfish Procambarus clarkii. Pigment cell research 12 1823925

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