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Items: 26


Mitochondrial Migraine: Disentangling the angiopathy paradigm in m.3243A>G patients.

Smeitink J, Koene S, Beyrath J, Saris C, Turnbull D, Janssen M.

JIMD Rep. 2019 Mar 14;46(1):52-62. doi: 10.1002/jmd2.12017. eCollection 2019 Mar.


A Drosophila Mitochondrial Complex I Deficiency Phenotype Array.

Foriel S, Renkema GH, Lasarzewski Y, Berkhout J, Rodenburg RJ, Smeitink JAM, Beyrath J, Schenck A.

Front Genet. 2019 Mar 27;10:245. doi: 10.3389/fgene.2019.00245. eCollection 2019.


Live-Imaging Readouts and Cell Models for Phenotypic Profiling of Mitochondrial Function.

Iannetti EF, Prigione A, Smeitink JAM, Koopman WJH, Beyrath J, Renkema H.

Front Genet. 2019 Mar 1;10:131. doi: 10.3389/fgene.2019.00131. eCollection 2019. Review.


Rescue from galactose-induced death of Leigh Syndrome patient cells by pyruvate and NAD.

Iannetti EF, Smeitink JAM, Willems PHGM, Beyrath J, Koopman WJH.

Cell Death Dis. 2018 Nov 14;9(11):1135. doi: 10.1038/s41419-018-1179-4.


The KHENERGY Study: Safety and Efficacy of KH176 in Mitochondrial m.3243A>G Spectrum Disorders.

Janssen MCH, Koene S, de Laat P, Hemelaar P, Pickkers P, Spaans E, Beukema R, Beyrath J, Groothuis J, Verhaak C, Smeitink J.

Clin Pharmacol Ther. 2019 Jan;105(1):101-111. doi: 10.1002/cpt.1197. Epub 2018 Sep 3.


KH176 Safeguards Mitochondrial Diseased Cells from Redox Stress-Induced Cell Death by Interacting with the Thioredoxin System/Peroxiredoxin Enzyme Machinery.

Beyrath J, Pellegrini M, Renkema H, Houben L, Pecheritsyna S, van Zandvoort P, van den Broek P, Bekel A, Eftekhari P, Smeitink JAM.

Sci Rep. 2018 Apr 26;8(1):6577. doi: 10.1038/s41598-018-24900-3.


Feeding difficulties, a key feature of the Drosophila NDUFS4 mitochondrial disease model.

Foriel S, Beyrath J, Eidhof I, Rodenburg RJ, Schenck A, Smeitink JAM.

Dis Model Mech. 2018 Mar 27;11(3). pii: dmm032482. doi: 10.1242/dmm.032482.


KH176 under development for rare mitochondrial disease: a first in man randomized controlled clinical trial in healthy male volunteers.

Koene S, Spaans E, Van Bortel L, Van Lancker G, Delafontaine B, Badilini F, Beyrath J, Smeitink J.

Orphanet J Rare Dis. 2017 Oct 16;12(1):163. doi: 10.1186/s13023-017-0715-0.


Therapeutic effects of the mitochondrial ROS-redox modulator KH176 in a mammalian model of Leigh Disease.

de Haas R, Das D, Garanto A, Renkema HG, Greupink R, van den Broek P, Pertijs J, Collin RWJ, Willems P, Beyrath J, Heerschap A, Russel FG, Smeitink JA.

Sci Rep. 2017 Sep 15;7(1):11733. doi: 10.1038/s41598-017-09417-5.


Hetero-oligomerization between the TNF receptor superfamily members CD40, Fas and TRAILR2 modulate CD40 signalling.

Smulski CR, Decossas M, Chekkat N, Beyrath J, Willen L, Guichard G, Lorenzetti R, Rizzi M, Eibel H, Schneider P, Fournel S.

Cell Death Dis. 2017 Feb 9;8(2):e2601. doi: 10.1038/cddis.2017.22.


Multiplexed high-content analysis of mitochondrial morphofunction using live-cell microscopy.

Iannetti EF, Smeitink JA, Beyrath J, Willems PH, Koopman WJ.

Nat Protoc. 2016 Sep;11(9):1693-710. doi: 10.1038/nprot.2016.094. Epub 2016 Aug 18.


Synthetic ligands of death receptor 5 display a cell-selective agonistic effect at different oligomerization levels.

Beyrath J, Chekkat N, Smulski CR, Lombardo CM, Lechner MC, Seguin C, Decossas M, Spanedda MV, Frisch B, Guichard G, Fournel S.

Oncotarget. 2016 Oct 4;7(40):64942-64956. doi: 10.18632/oncotarget.10508.


Mitochondrial disorders in children: toward development of small-molecule treatment strategies.

Koopman WJ, Beyrath J, Fung CW, Koene S, Rodenburg RJ, Willems PH, Smeitink JA.

EMBO Mol Med. 2016 Apr 1;8(4):311-27. doi: 10.15252/emmm.201506131. Review.


Statin-Induced Myopathy Is Associated with Mitochondrial Complex III Inhibition.

Schirris TJ, Renkema GH, Ritschel T, Voermans NC, Bilos A, van Engelen BG, Brandt U, Koopman WJ, Beyrath JD, Rodenburg RJ, Willems PH, Smeitink JA, Russel FG.

Cell Metab. 2015 Sep 1;22(3):399-407. doi: 10.1016/j.cmet.2015.08.002.


Toward high-content screening of mitochondrial morphology and membrane potential in living cells.

Iannetti EF, Willems PH, Pellegrini M, Beyrath J, Smeitink JA, Blanchet L, Koopman WJ.

Int J Biochem Cell Biol. 2015 Jun;63:66-70. doi: 10.1016/j.biocel.2015.01.020. Epub 2015 Feb 8. Review.


Mitochondrial diseases: Drosophila melanogaster as a model to evaluate potential therapeutics.

Foriel S, Willems P, Smeitink J, Schenck A, Beyrath J.

Int J Biochem Cell Biol. 2015 Jun;63:60-5. doi: 10.1016/j.biocel.2015.01.024. Epub 2015 Feb 7. Review.


Quantifying small molecule phenotypic effects using mitochondrial morpho-functional fingerprinting and machine learning.

Blanchet L, Smeitink JA, van Emst-de Vries SE, Vogels C, Pellegrini M, Jonckheere AI, Rodenburg RJ, Buydens LM, Beyrath J, Willems PH, Koopman WJ.

Sci Rep. 2015 Jan 26;5:8035. doi: 10.1038/srep08035.


Multidrug resistance protein 4/ ATP binding cassette transporter 4: a new potential therapeutic target for acute myeloid leukemia.

Copsel S, Bruzzone A, May M, Beyrath J, Wargon V, Cany J, Russel FG, Shayo C, Davio C.

Oncotarget. 2014 Oct 15;5(19):9308-21.


Cysteine-rich domain 1 of CD40 mediates receptor self-assembly.

Smulski CR, Beyrath J, Decossas M, Chekkat N, Wolff P, Estieu-Gionnet K, Guichard G, Speiser D, Schneider P, Fournel S.

J Biol Chem. 2013 Apr 12;288(15):10914-22. doi: 10.1074/jbc.M112.427583. Epub 2013 Mar 5.


Antitumor activity of liposomal ErbB2/HER2 epitope peptide-based vaccine constructs incorporating TLR agonists and mannose receptor targeting.

Thomann JS, Heurtault B, Weidner S, BrayƩ M, Beyrath J, Fournel S, Schuber F, Frisch B.

Biomaterials. 2011 Jul;32(20):4574-83. doi: 10.1016/j.biomaterials.2011.03.015. Epub 2011 Apr 7.


A simple approach to cancer therapy afforded by multivalent pseudopeptides that target cell-surface nucleoproteins.

Destouches D, Page N, Hamma-Kourbali Y, Machi V, Chaloin O, Frechault S, Birmpas C, Katsoris P, Beyrath J, Albanese P, Maurer M, Carpentier G, Strub JM, Van Dorsselaer A, Muller S, Bagnard D, Briand JP, Courty J.

Cancer Res. 2011 May 1;71(9):3296-305. doi: 10.1158/0008-5472.CAN-10-3459. Epub 2011 Mar 17.


Ligand dimerization programmed by hybridization to study multimeric ligand-receptor interactions.

Gorska K, Beyrath J, Fournel S, Guichard G, Winssinger N.

Chem Commun (Camb). 2010 Nov 7;46(41):7742-4. doi: 10.1039/c0cc02852e. Epub 2010 Sep 20.


Replacement of Ala by Aib improves structuration and biological stability in thymine-based alpha-nucleopeptides.

Geotti-Bianchini P, Moretto A, Peggion C, Beyrath J, Bianco A, Formaggio F.

Org Biomol Chem. 2010 Mar 21;8(6):1315-21. doi: 10.1039/b920211k. Epub 2010 Jan 21.


Novel powerful water-soluble lipid immunoadjuvants inducing mouse dendritic cell maturation and B cell proliferation using TLR2 pathway.

Spanedda MV, Heurtault B, Weidner S, Baehr C, Boeglin E, Beyrath J, Milosevic S, Bourel-Bonnet L, Fournel S, Frisch B.

Bioorg Med Chem Lett. 2010 Mar 15;20(6):1869-72. doi: 10.1016/j.bmcl.2010.01.146. Epub 2010 Feb 2.


Multivalent DR5 peptides activate the TRAIL death pathway and exert tumoricidal activity.

Pavet V, Beyrath J, Pardin C, Morizot A, Lechner MC, Briand JP, Wendland M, Maison W, Fournel S, Micheau O, Guichard G, Gronemeyer H.

Cancer Res. 2010 Feb 1;70(3):1101-10. doi: 10.1158/0008-5472.CAN-09-2889. Epub 2010 Jan 26.


Design and synthesis of intrinsically cell-penetrating nucleopeptides.

Geotti-Bianchini P, Beyrath J, Chaloin O, Formaggio F, Bianco A.

Org Biomol Chem. 2008 Oct 21;6(20):3661-3. doi: 10.1039/b811639c. Epub 2008 Sep 17.


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