Artykuły
----------2022----------
C19orf12
ablation causes ferroptosis in mitochondrial membrane protein-associated with
neurodegeneration Changjuan Shao, Julia Zhu,
Xiaopin Ma, Sandra L. Siedlak, Mark L. Cohen, Alan Lerner, Wenzhang Wang,
"Free Radical Biology and Medicine", February 2022
Generation
of two human iPSC lines, HMGUi003-A and MRIi028-A, carrying pathogenic
biallelic variants in the PPCS gene Arcangela
Iuso, Fangfang Zhang, Ejona Rusha, Birgit Campbell, Tatjana Dorn, Enrica
Zanuttigh, Dorothea Haasf, Yair Anikster, Gabriele Lederer, Anna Pertek,
Polyxeni Nteli, Karl-Ludwig Laugwitz, Alessandra Moretti, "Stem Cell
Research", March 2022
Investigating
demyelination, efficient remyelination and remyelination failure in organotypic
cerebellar slice cultures: Workflow and practical tips Rianne
P. Gorter, Naomi S. Dijksman, Wia Baron, and Holly Colognato, "Methods in
Cell Biology", March 2022
Nazo,
the Drosophila homolog of the NBIA-mutated protein – c19orf12, is required for
triglyceride homeostasis Perinthottathil
Sreejith, Sara Lolo, Kristen R. Patten, Maduka Gunasinghe, Neya More, Leo J.
Pallanck, Rajnish Bharadwaj, BioRxiv, September 2022
Vitamin
E prevents lipid peroxidation and iron accumulation in PLA2G6-Associated
Neurodegeneration Irene Villalon,
Mónica Álvarez Códoba, Suleva Povea-Cabello, Juan Miguel Suárez-Rivero,
"Neurobiology of Disease", February 2022
----------2021----------
----------2015----------
----------2011----------
----------2021----------
Activation
of Nrf2 signaling pathway by natural and synthetic chalcones: a therapeutic
road map for oxidative stress Melford Chuka
Egbujor, Sarmistha Saha, Brigitta Buttari, Elisabetta Profumo, Luciano Saso,
"Expert Reviev of Clinical Pharmacology", March
2021
Antipsychotic
drugs counteract autophagy and mitophagy in multiple sclerosis Simone
Patergnani, Massimo Bonora, Selene Ingusci, Maurizio Previati, Saverio Marchi,
Silvia Zucchini, Mariasole Perrone, Mariusz R. Wieckowski, Massimiliano
Castellazzi, Maura Pugliatti, Carlotta Giorgi, Michele Simonato, Paolo Pinton,
"Proceedings of the National Academy of Sciences", June 2021
DNA
methylation and lipid metabolism: an EWAS of 226 metabolic measures Monica
del C. Gomez Alonso, Anja Kretschmer, Rory Wilson, Liliane Pfeifer, Ville
Karhunen, Ilkka Seppälä, Weihua Zhang, Kirstin Mittelstraß, Simone Wahl, Pamela
R. Matias Garcia, Holger Prokisch, Sacha Horn, Thomas Meitinger, Luis R.
Serrano Garcia, Sylvain Sebert, Olli Raitakari, Marie Loh, Wolfgang Rathmann,
Martina Müller Nurasyid, Christian Herder, Michael Roden, Mikko Hurme, Marjo
Riitta Jarvelin, Mika Ala Korpela, Jaspal S. Kooner, Annette Peters, Terho
Lehtimäki , John C. Chambers, Christian Gieger, Johannes Kettunen, Melanie
Waldenberger, "Clinical Epigenetics", January 2021
Emerging
Disease-Modifying Therapies in Neurodegeneration With Brain Iron Accumulation
(NBIA) Disorders Vassilena Iankova,
Ivan Karin, Thomas Klopstock, Susanne A. Schneider, "Frontiers in
Neurology", April 2021
Retrospective
analysis of 17 patients with mitochondrial membrane protein-associated
neurodegeneration diagnosed in Russia Peter
Sparber, Tatiana Krylova, Svetlana Repina, Nina Demina, Galina Rudenskaya, Inna
Sharkova, Artem Sharkov, Vitaly Kadyshev, Ilya Kanivets, Sergey Korostelev,
Ekaterina Pomerantseva, Vladimir Kaimonov, Svetlana Mikhailova, Ekaterina
Zakharova, Mikhail Skoblov, "Parkinsonism & Related Disorders",
February 2021
Role
of Nrf2 in Synaptic Plasticity and Memory in Alzheimer’s Disease Don
A. Davies, Aida Adlimoghaddam, Benedict C. Albensi, "Cells",
July 2021
RT001
in Progressive Supranuclear Palsy—Clinical and In-Vitro
Observations Plamena R.
Angelova, Kristin M. Andruska, Mark G. Midei, Mario Barilani, Paldeep Atwal,
Oliver Tucher, Peter Milner, Frederic Heerinckx, Mikhail S. Shchepinov,
"Antioxidants", June 2021
The
mitochondrial permeability transition pore: an evolving concept critical for
cell life and death Giampaolo Morciano,
Natalia Naumova, Piotr Koprowski, Sara Valente, Vilma A. Sard, Yaiza Potes,
Alessandro Rimessi, Mariusz R. Wieckowski and Paulo J. Oliveira,
"Biological Reviews", June 2021
Update
on the Histoenzymatic Methods for Visualization of the Activity of Individual
Mitochondrial Respiratory Chain Complexes in the Human Frozen Sections Wieckowski
M.R., Pronicki M., Karkucinska-Wieckowska A., "Methods in Molecular
Biology", 2021
WDR45,
one gene associated with multiple neurodevelopmental disorders Yingying
Cong, Vincent So, Marina A J Tijssen, Dineke S Verbeek, Fulvio Reggiori, Mario
Mauthe, "Autophagy", April 2021
----------2020----------
A bacterial cytidine deaminase toxin enables
CRISPR-free mitochondrial base editing Beverly Y. Mok, Marcos H. de Moraes, Jun Zeng,
Dustin E. Bosch, Anna V. Kotrys, Aditya Raguram, FoSheng Hsu, Matthew C. Radey,
S. Brook Peterson, Vamsi K. Mootha, Joseph D. Mougous, David R. Liu,
"Nature", July 2020
A
High-Density Human Mitochondrial Proximity Interaction Network Hana
Antonicka, Zhen-Yuan Lin, Alexandre Janer, Mari J. Aaltonen, Woranontee
Weraarpachai, Anne-Claude Gingras, and Eric A. Shoubridge, "Cell
Metabolism", September 2020
A
New Current for the Mitochondrial Permeability Transition, Massimo
Bonora and Paolo Pinton,"Trends in Biochemical Sciences", July
2019
Antioxidant and Anti-Inflammatory Effect of Nrf2 Inducer
Dimethyl Fumarate in Neurodegenerative Diseases Sarah
A. Scuderi, Alessio Ardizzone, Irene Paterniti, Emanuela Esposito, Michela
Campolo, "Antioxidants", July 2020
Azoramide
protects iPSC-derived dopaminergic neurons with PLA2G6 D331Y mutation through
restoring ER function and CREB signaling Minjing
Ke, Cheong-Meng Chong, Haitao Zeng, Miaodan Huang, Zhijian Huang, Ke Zhang,
Xiaotong Cen, Jia-Hong Lu , Xiaoli Yao, Dajiang Qin, Huanxing Su, "Cell
Death & Disease", February 2020
Harmful
Iron-Calcium Relationship in Pantothenate kinase Associated
Neurodegeneration Paolo Santambrogio,
Maddalena Ripamonti, Chiara Paolizzi, Celeste Panteghini, Miryam Carecchio,
Luisa Chiapparini, Marzia Raimondi, Alicia Rubio, Ivano Di Meo, Anna Cozzi,
Stefano Taverna, Giuseppe De Palma, Valeria Tiranti Sonia, Levi,
"International Journal of Molecular Sciences", May 2020
Mitochondria
and lipid peroxidation in the mechanism of neurodegeneration: Finding ways for
prevention Plamena R. Angelova, Noemi Esteras, Andrey Y. Abramov,
"Medicinal Research Reviews", July 2020
Mitochondrial
Dysfunction, Oxidative Stress, and Neuroinflammation: Intertwined Roads to
Neurodegeneration Anna Picca, Riccardo Calvani, Hélio José Coelho‐Júnior, Francesco
Landi, Roberto Bernabei, Emanuele Marzetti, "Antioxidants",
July 2020
Neuronal
Ablation of CoA Synthase Causes Motor Deficits, Iron Dyshomeostasis, and
Mitochondrial Dysfunctions in a CoPAN Mouse Model Ivano
Di Meo, Chiara Cavestro, Silvia Pedretti, Tingting Fu, Simona Ligorio,
Antonello Manocchio, Lucrezia Lavermicocca, Paolo Santambrogio, Maddalena
Ripamonti, Sonia Levi, Sophie Ayciriex, Nico Mitro, Valeria Tiranti,
"International Journal of Molecular Sciences", December 2020
NDUFS4
deletion triggers loss of NDUFA12 in Ndufs4-/- mice and Leigh syndrome
patients: A stabilizing role for NDUFAF2 Adjobo-Hermans;
Ria de Haas; Peter H Willems; Aleksandra Wojtala; Sjenet E van Emst-de Vries;
Jori A Wagenaars; Mariel van den Brand; Richard J Rodenburg; Jan A Smeitink;
Leo G Nijtmans; Leonid A Sazanov; Mariusz R Wieckowski, Werner J Koopman, "Biochimica
et Biophysica Acta– Bioenergetics", April 2020
Omega 3 versus Omega 6 polyunsaturated fatty acids in cardio-metabolic health Halim M. and Halim A., "Journal of Health Care and Research", June 2020
Omega 3 versus Omega 6 polyunsaturated fatty acids in cardio-metabolic health Halim M. and Halim A., "Journal of Health Care and Research", June 2020
Revisiting
the blood-brain barrier: A hard nut to crack in the transportation of drug
molecules Seetha Harilal, Jobin Jose,
Della Grace Thomas Parambi, Rajesh Kumar, Mazhuvancherry Kesavan Unnikrishnan,
Md. Sahab Uddin, Githa Elizabeth Mathew, Rahul Pratap, Akash Marathakam, Bijo
Mathew, "Brain Research Bulletin", April
2020
The
mitophagy receptor FUN14 domain-containing 1 (FUNDC1): A promising biomarker
and potential therapeutic target of human diseases”
Weilin Zhang, "Genes & Diseases", September 2020
The
Downregulation of c19orf12 Negatively Affects Neuronal and Musculature
Development in Zebrafish Embryos Luca
Mignani, Daniela Zizioli, Giuseppe Borsani, Eugenio Monti and Dario Finazzi,
"Frontiers in Cell and Developmental Biology", December 2020
Treatment of infantile neuroaxonal dystrophy with RT001: A di-deuterated
ethyl ester of linoleic acid: Report of two cases Darius Adams,
Mark Midei, Jahannaz Dastgir, Christina Flora, Robert J Molinari, Frederic
Heerinckx, Sarah Endemann, Paldeep Atwal, Peter Milner, Mikhail S. Shchepinov,
"JIMD Reports", March 2020
When It Comes to an End: Oxidative Stress Crosstalk with Protein Aggregation and Neuroinflammation Induce Neurodegeneration Patrycja Michalska, Rafael Leon, "Antioxidants", August 2020
When It Comes to an End: Oxidative Stress Crosstalk with Protein Aggregation and Neuroinflammation Induce Neurodegeneration Patrycja Michalska, Rafael Leon, "Antioxidants", August 2020
----------2019----------
Fine-tuning
of the respiratory complexes stability and supercomplexes assembly in cells
defective of complex III Concetta V.
Tropeano, Serena J. Aleo, Claudia Zanna, Marina Roberti, Letizia Scandiffio,
Paola Loguercio Polosa, Jessica Fiori, Emanuele Porru, Aldo Roda, Valerio
Carelli, Stefan Steimle, Fevzi Daldal, Michela Rugolo, Anna Ghelli,
"Biochimica et Biophysica Acta", December 2019
Myelin repair stimulated by CNS-selective thyroid hormone
action Meredith D. Hartley, Tania Banerji, Ian J. Tagge,
Lisa L. Kirkemo, Priya Chaudhary, Evan Calkins, Danielle Galipeau, Mitra D.
Shokat, Margaret J. DeBell, Shelby Van Leuven, Hannah Miller, Gail Marracci,
Edvinas Pocius, Tapasree Banerji, Skylar J. Ferrara, J. Matthew Meinig, Ben
Emery, Dennis Bourdette, Thomas S. Scanlan, "JCI Insight", April 2019
Nrf2
jako terapeutyczny punkt uchwytu w profilaktyce i terapii chorób
cywilizacyjnych Marta Cykowiak, Violetta
Krajka-Kuźniak, "Farmacja współczesna", 2019
NRF2
plays a critical role in mitigating lipid peroxidation and ferroptosis Matthew
Dodson, Raul Castro-Portuguez, Donna D. Zhang, "Redox Biology", May
2019
Parkinson’s disease-associated iPLA2-VIA/PLA2G6 regulates neuronal
functions and α-synuclein stability through membrane remodeling Akio
Moria, Taku Hatanoa, Tsuyoshi Inoshita, Kahori Shiba-Fukushima, Takahiro
Koinuma, Hongrui Meng, Shin-Ichiro Kubo, Spencer Spratt, Changxu Cui, Chikara
Yamashita, Yoshimi Miki, Kei Yamamoto, Tetsuya Hirabayashi, Makoto Murakami,
Yoshikazu Takahashi, Hideo Shindou, Takashi Nonaka, Masato Hasegawa, Ayami
Okuzumi, Yuzuru Imai, Nobutaka Hattori, "Proceedings of the National
Academy of Sciences", September 2019
Silymarin’s
Inhibition and Treatment Effects for Alzheimer’s Disease,
Hong Guo, Hui Cao, Xiaowei Cui, Wenxiu Zheng, Shanshan Wang, Jiyang Yu and Zhi
Chen, "Molecules", May 2019
Sulfoaphane
- role in aging and neurodegeneration Roberto
Santín-Márquez, Adriana Alarcón-Aguilar, Norma Edith López-Diazguerrero, Niki
Chondrogianni, Mina Königsberg, "GeroScience", April 2019
----------2018----------
Mitochondrial
alterations accompanied by oxidative stress conditions in skin fibroblasts of
Huntington's disease patients Paulina
Jędrak, Paweł Mozolewski, Grzegorz Węgrzyn and Mariusz R. Więckowski, "Metabolic Brain Disease", December 2018
Recovering
mitochondrial function in patients’ fibroblasts Mariusz
R. Wieckowski, Alberto Danese, Giampaolo Morciano, Maciej Pronicki, Jerzy
Duszynski, Paolo Pinton and Agnieszka Karkucinska-Wieckowska, "Mitochondrial Biology and Experimental Therapeutics", March 2018
The role of Nrf2 signaling in counteracting
neurodegenerative diseases Albena T.
Dinkova-Kostova, Rumen V. Kostov and Aleksey G. Kazantsev, "The FEBS
Journal", October 2018
----------2017----------
Bioactivity
of Olive Oil Phenols in Neuroprotection Cristina
Angeloni, Marco Malaguti, Maria Cristina Barbalace, Silvana Hrelia,
"International Journal of Molecular Sciences", October 2017
Modulation
of mitochondrial dysfunction-related oxidative stress in fibroblasts of
patients with Leigh syndrome by inhibition of prooxidative p66Shc pathway Aleksandra
Wojtala, Agnieszka Karkucinska-Wieckowska, Vilma A Sardao, Joanna
Szczepanowska, Pawel Kowalski, Maciej Pronicki, Jerzy Duszynski and Mariusz R.
Wieckowski, " Mitochondrion", July 2017
----------2016----------
Astaksantyna
– karotenoidowy przeciwutleniacz o właściwościach kardioprotekcyjnych
Astaxanthin – a carotenoid antioxidant with cardioprotective properties Małgorzata
Sieradzka, Joanna Kołodziejczyk-Czepas, "Problemy Higieny
i Epidemiologii", 2016
Insulin-like
growth factor 1 (IGF-1) therapy: Mitochondrial dysfunction and
diseases M.C. Sádaba, I. Martín-Estal,
J.E. Puche, I. Castilla-Cortázar, "Biochimica et Biophysica Acta", March 2016
----------2015----------
Histoenzymatic methods for visualization of the activity of individual mitochondrial
respiratory chain complexes in the muscle biopsies from patients with
mitochondrial defects Agnieszka
Karkucinska-Wieckowska, Maciej Pronicki and Mariusz R. Wieckowski, "Methods Molecular Biology", 2015
Mutations
of C19orf12, coding for a transmembrane glycine zipper containing mitochondrial
protein, cause mis-localization of the protein, inability to respond to
oxidative stress and increased mitochondrial Ca2+ Paola
Venco, Massimo Bonora, Carlotta Giorgi, Elena Papaleo, Arcangela Iuso, Holger
Prokisch, Paolo Pinton and Valeria Tiranti, "Frontiers in Genetics",
May 2015
Role
of 5-hydroxymethylcytosine in neurodegeneration Shariq
I. Sherwani, Haseeb A. Khan, "Gene", October 2015
----------2013----------
----------2013----------
Disrupted
ATP synthase activity and mitochondrial hyperpolarisation-dependent oxidative
stress is associated with p66Shc phosphorylation in fibroblasts of NARP patients Lebiedzinska
M, Karkucinska-Wieckowska A, Wojtala A, Suski JM, Szabadkai G, Wilczynski G,
Wlodarczyk J, Diogo CV, Oliveira PJ, Tauber J, Ježek P, Pronicki M, Duszynski
J, Pinton P, Wieckowski MR, "The International Journal of Biochemistry &
Cell Biology", January 2013
----------2012----------
Mitochondrial
calcium homeostasis as potential target for mitochondrial medicine C,
Agnoletto C, Bononi A, Bonora M, De Marchi E, Marchi S, Missiroli S, Patergnani
S, Poletti F, Rimessi A, Suski JM, Wieckowski MR, Pinton P, "Mitochondrion", January 2012
----------2011----------
Increased
reactive oxygen species (ROS) production and low catalase level in fibroblasts
of a girl with MEGDEL association (Leigh syndrome, deafness, 3-methylglutaconic
aciduria) Karkucinska-Wieckowska A, Lebiedzinska M,
Jurkiewicz E, Pajdowska M, Trubicka J, Szymanska-Debinska T, Suski J, Pinton P,
Duszynski J, Pronicki M, Wieckowski MR, Pronicka E, "Folia
Neuropathologica", March 2011
----------2010----------
Oxidative
stress-dependent p66Shc phosphorylation in skin fibroblasts of children with
mitochondrial disorders Lebiedzinska M,
Karkucinska-Wieckowska A, Giorgi C, Karczmarewicz E, Pronicka E, Pinton P,
Duszynski J, Pronicki M and Wieckowski MR, "Biochimica et Biophysica
Acta – Bioenergetics", March 2010
----------2004----------
Glutation
: biosynteza, czynniki indukujące oraz stężenie w wybranych jednostkach
chorobowych Bożena Bukowska, "Medycyna
Pracy", 2004