| Unveiling the clinical and electrophysiological profile of CMTX6: Insights from two Brazilian families. | Unveiling the clinical and electrophysiological profile of CMTX6: Insights from two Brazilian families.
Maciel VAZ, Maximiano-Alves G, Frezatti RSS, Alves ALM, Andrade BMA, Leal RCC, Tomaselli PJ, Reilly MM, Marques W. | 12/27/2023 |
| Charcot-Marie-tooth disease causing mutation (p.R158H) in pyruvate dehydrogenase kinase 3 (PDK3) affects synaptic transmission, ATP production and causes neurodegeneration in a CMTX6 C. elegans model. | Charcot-Marie-tooth disease causing mutation (p.R158H) in pyruvate dehydrogenase kinase 3 (PDK3) affects synaptic transmission, ATP production and causes neurodegeneration in a CMTX6 C. elegans model.
Narayanan RK, Brewer MH, Perez-Siles G, Ellis M, Ly C, Burgess A, Neumann B, Nicholson GA, Vucic S, Kennerson ML., Free PMC Article | 04/16/2022 |
| Overexpression of PDK2 and PDK3 reflects poor prognosis in acute myeloid leukemia. | Overexpression of PDK2 and PDK3 reflects poor prognosis in acute myeloid leukemia.
Cui L, Cheng Z, Liu Y, Dai Y, Pang Y, Jiao Y, Ke X, Cui W, Zhang Q, Shi J, Fu L. | 05/15/2021 |
| Energy metabolism and mitochondrial defects in X-linked Charcot-Marie-Tooth (CMTX6) iPSC-derived motor neurons with the p.R158H PDK3 mutation. | Energy metabolism and mitochondrial defects in X-linked Charcot-Marie-Tooth (CMTX6) iPSC-derived motor neurons with the p.R158H PDK3 mutation.
Perez-Siles G, Cutrupi A, Ellis M, Screnci R, Mao D, Uesugi M, Yiu EM, Ryan MM, Choi BO, Nicholson G, Kennerson ML., Free PMC Article | 12/12/2020 |
| Effect of pH on the structure and function of pyruvate dehydrogenase kinase 3: Combined spectroscopic and MD simulation studies. | Effect of pH on the structure and function of pyruvate dehydrogenase kinase 3: Combined spectroscopic and MD simulation studies.
Anwar S, Kar RK, Haque MA, Dahiya R, Gupta P, Islam A, Ahmad F, Hassan MI. | 11/28/2020 |
| miR-497-5p inhibits gastric cancer cell proliferation and growth through targeting PDK3. | miR-497-5p inhibits gastric cancer cell proliferation and growth through targeting PDK3.
Feng L, Cheng K, Zang R, Wang Q, Wang J., Free PMC Article | 09/19/2020 |
| PDK3 forms a positive feedback loop with HSF1 to drive glycolysis in chemoresistance. | Metabolic enzyme PDK3 forms a positive feedback loop with transcription factor HSF1 to drive chemoresistance.
Xu J, Shi Q, Xu W, Zhou Q, Shi R, Ma Y, Chen D, Zhu L, Feng L, Cheng AS, Morrison H, Wang X, Jin H., Free PMC Article | 06/20/2020 |
| The pyruvate dehydrogenase kinases (PDKs) PDK1 and PDK3 are direct targets of KDM4A and E2F1 and modulate the switch between glycolytic metabolism and mitochondrial oxidation. | KDM4A Coactivates E2F1 to Regulate the PDK-Dependent Metabolic Switch between Mitochondrial Oxidation and Glycolysis.
Wang LY, Hung CL, Chen YR, Yang JC, Wang J, Campbell M, Izumiya Y, Chen HW, Wang WC, Ann DK, Kung HJ., Free PMC Article | 11/18/2017 |
| We have identified a p.R158H PDK3 mutation in a Korean X-linked dominant Charcot-Marie-Tooth type 6 family | X-linked Charcot-Marie-Tooth disease type 6 (CMTX6) patients with a p.R158H mutation in the pyruvate dehydrogenase kinase isoenzyme 3 gene.
Kennerson ML, Kim EJ, Siddell A, Kidambi A, Kim SM, Hong YB, Hwang SH, Chung KW, Choi BO. | 12/17/2016 |
| Accordingly, elevated levels of PDK1, PDK3, and PKM2 and reduced PK activity could be observed in iPSCs and human embryonic stem cells in the undifferentiated state | HIF1α modulates cell fate reprogramming through early glycolytic shift and upregulation of PDK1-3 and PKM2.
Prigione A, Rohwer N, Hoffmann S, Mlody B, Drews K, Bukowiecki R, Blümlein K, Wanker EE, Ralser M, Cramer T, Adjaye J., Free PMC Article | 09/20/2014 |
| Reduced pyruvate flux due to R158H mutant PDK3-mediated hyper-phosphorylation of the pyruvate dehydrogenase complex is the underlying pathogenic cause of peripheral neuropathy in X-linked dominant Charcot-Marie-Tooth disease. | A new locus for X-linked dominant Charcot-Marie-Tooth disease (CMTX6) is caused by mutations in the pyruvate dehydrogenase kinase isoenzyme 3 (PDK3) gene.
Kennerson ML, Yiu EM, Chuang DT, Kidambi A, Tso SC, Ly C, Chaudhry R, Drew AP, Rance G, Delatycki MB, Züchner S, Ryan MM, Nicholson GA., Free PMC Article | 08/31/2013 |
| Findings suggest the HIF-1/PDK3 bioenergetic pathway as a new target for therapeutic intervention in metastatic melanoma. | Inactivation of the HIF-1α/PDK3 signaling axis drives melanoma toward mitochondrial oxidative metabolism and potentiates the therapeutic activity of pro-oxidants.
Kluza J, Corazao-Rozas P, Touil Y, Jendoubi M, Maire C, Guerreschi P, Jonneaux A, Ballot C, Balayssac S, Valable S, Corroyer-Dulmont A, Bernaudin M, Malet-Martino M, de Lassalle EM, Maboudou P, Formstecher P, Polakowska R, Mortier L, Marchetti P. | 12/22/2012 |
| increased PDK3 expression due to elevated HIF-1alpha in cancer cells may play critical roles in metabolic switch during cancer progression and chemoresistance in cancer therapy | Induction of pyruvate dehydrogenase kinase-3 by hypoxia-inducible factor-1 promotes metabolic switch and drug resistance.
Lu CW, Lin SC, Chen KF, Lai YY, Tsai SJ., Free PMC Article | 01/21/2010 |
| Distinct structural mechanisms for inhibition of PDK3 by AZD7545, dichloroacetate, and radicol. | Distinct structural mechanisms for inhibition of pyruvate dehydrogenase kinase isoforms by AZD7545, dichloroacetate, and radicicol.
Kato M, Li J, Chuang JL, Chuang DT., Free PMC Article | 01/21/2010 |
| PDK2, PDK3 and PDK4 are primary PPARbeta/delta target genes in humans underlining the importance of the receptor in the control of metabolism | Three members of the human pyruvate dehydrogenase kinase gene family are direct targets of the peroxisome proliferator-activated receptor beta/delta.
Degenhardt T, Saramäki A, Malinen M, Rieck M, Väisänen S, Huotari A, Herzig KH, Müller R, Carlberg C. | 01/21/2010 |
| crystal structure of the asymmetric PDHK3/lipoyl domain 2 complex; data suggest that the asymmetric complex represents a physiological state in which binding of a single L2-domain activates one of the PDHK protomers while inactivating another | Crystal structure of an asymmetric complex of pyruvate dehydrogenase kinase 3 with lipoyl domain 2 and its biological implications.
Devedjiev Y, Steussy CN, Vassylyev DG., Free PMC Article | 01/21/2010 |
| analysis of residues in L2 and residues in the C-terminal region and the lipoyl-binding pocket of PDK3 which are critical determinants for the cross-talk between L2 and PDK3, which up-regulates PDK3 activity | Structural determinants for cross-talk between pyruvate dehydrogenase kinase 3 and lipoyl domain 2 of the human pyruvate dehydrogenase complex.
Tso SC, Kato M, Chuang JL, Chuang DT. | 01/21/2010 |
| mRNA levels not changed in skeletal muscle during fasting | Pyruvate dehydrogenase activation and kinase expression in human skeletal muscle during fasting.
Spriet LL, Tunstall RJ, Watt MJ, Mehan KA, Hargreaves M, Cameron-Smith D. | 01/21/2010 |