Laboratory of Mitochondrial Biology and Metabolism
Merry CHristmas
LAB NEWS
Our MitoLab team at the at the 64th International Conference of the Bioscience of Lipids (ICBL 2024) October 15-19 in Taipei, Taiwan
prof. Mariusz Wieckowski presented a talk "The impact of omega 3-6-9 fatty acids treatment on proteome of lipid metabolism-related pathways in fibroblasts from patients with Neurodegeneration with Brain Iron Accumulation (NBIA)".
Barbara Pakuła, MSc, a PhD student in the MitoLab presented a poster "The impact of lipid accumulation on the redox status in a cellular model depicting the progression from steatosis to steatosis with inflammation".
Agata Wydrych, MSc, a PhD student in the MitoLab presented a poster "The impact of omega 3-6-9 fatty acids treatment on proteome of lipid metabolism-related pathways in fibroblasts from patients with Neurodegeneration with Brain Iron Accumulation (NBIA)".
Working meeting in Kraków with Prof. Werner Koopman from Radboud University Medical Center, Nijmegen
September 19-20, 2024 our MitoLab team organized "working meeting" in Kraków with prof. Werner Koopman where we discussed progress in our recent projects on rare diseases.
Our MitoLab team at
"Unlocking Hope for the Ultra-rare" conference"
From 4 to 5 September 2024 we participated in an extremely important event for PACS2 community. It was First International Conference organised by PACS2 Research Foundation and Center of Excellence for Rare and Unknown Disorders of Medical University of Warsaw. It was great to meet all these wonderful people involved in helping patients and their families by investigating pathogenesis of PACS2 Syndrome.
Durng the conference I gave a talk entitled: "PACS2 patients: what is wrong with their metabolism - looking for metabolic alterations in PACS2 syndrome using patients' fibroblasts" where I summarized the progress in the study performed by MitoLab team and the team from Laboratory of Cell Signaling and Metabolic Disorders.
Our MitoLab team at the 22nd European Bioenergetics Conference:
"What is life?"
From 26th to 31st of August 2024 we actively participated in 22nd Annual European Bioenergetics. I was invited to give the talk entitled "Metabolic dysfunction in fibroblasts derived from patients with mitochondrial membrane protein-associated neurodegeneration - hope for pharmacological treatment?" Moreover, other members of our MitoLab: Magdalena Lebiedzinska-Arciszewska, Barbara Pakuła and Agata Wydrych presented their work during the poster session.
Our MitoLab team at: "Technical and Scientific Workshops" meeting organized by NBIA Polska Association
On June 15, 2024, dr Magdalena Lebiedzinska-Arciszewska gave the talk entitled: "Summary of the "Science for Society" project and research in the field of NBIA" during "Technical and Scientific Workshops" organized by NBIA Polska Association. During her talk, dr Magdalena Lebiedzinska-Arciszewska summarized the the study carried out in Nencki Institute.
Meeting was addressed to families and patients with NBIA.
PhD student Barbara Pakuła with award for outstanding oral presentation at ESCI conference
Barbara Pakuła, MSc, a PhD student in the MitoLab, won the award for outstanding oral presentation during 58th Annual Scientific Meeting of the European Society for Clinical Investigation in the "Mitochondria" session.
Title of presentation: "Autophagy and MASLD progression - from cellular model to patients’ liver biopsies".
Our MitoLab team at the 58th ESCI conference:
"Connecting the dots. The power of integrative medicine"
From 5th to 7th of June 2024 we actively participated in 58th Annual Scientific Meeting of the European Society for Clinical Investigation.
During "Mitochondrial Diseases Session" prof. Mariusz Wieckowski presented a talk "MPAN patients: what is wrong with their metabolism - discovering bioenergetic alterations in NBIA disease".
During "Mitochondrial Diseases Session" Barbara Pakuła, MSc, a PhD student in the MitoLab presented a talk "Autophagy and MASLD progression - from cellular model to patients’ liver biopsies".
During "Mitochondrial Diseases Session" Agata Wydrych, MSc, a PhD student in the MitoLab presented a talk "Ways to improve impaired metabolism in MPAN patients - is there a hope for pharmacological intervention based on an in vitro study".
Our MitoLab team at the conference:
"Focus on Lipid Metabolism"
From 23rd to 24th of May 2024 we participated in an extremely important event for our Metabolic group. This was the first International Conference for Life Sciences "Focus on Lipid Metabolism" organized by Nencki Institute.
Link to the conference WebSite: https://conference.nencki.edu.pl/
Symposium: „Advances in muscle and exercise physiology"
On May 17th 2024, I had the opportunity to present the results we’ve gathered during two years study in our laboratory on NBIA-MPAN disease at the Jagiellonian University Medical College.
Official visits at the University of Gdańsk and Medical University of Gdańsk in the range of NBIA project
On April 15th 2024 during the meeting with Prof. Grzegorz Węgrzyn at the University of Gdańsk MitoLab team resented new findings in the NBIA field.
Next, MitoLab team visited Faculty of Medicine at the Medical Univeristy of Gdańsk where we had a meeting with Prof. Magdalena Ponikowska and Prof. Michał Woźniak team.
Brain Week 2024 at the Nencki Institute: "From the laboratory to the sickbed", March 11-16, 2024.
On March 14th 2024 during World Brain Week, I had the opportunity to give the talk entitled "Rare diseases and the struggles of scientists - what is NBIA and PACS2 syndrome".
Our MitoLab team at the Rare Disease Day Conference
On February 29th 2024, which was the World Rare Disease Day - I had the opportunity to give the talk entitled "Research towards understanding the pathomechanism of neurodegeneration with iron storage (NBIA)" during Rare Disease Day Conference co-organised by the Institute of Biochemistry and Biophysics PAS.
Official visit at the University of Coimbra
On December 12th 2023 we had a very fruitful meeting in the Senate Hall of the University of Coimbra.
Together with vice Rector Joao Ramalho-Santos, we established a strategic plan for scientific cooperation between the Nencki Institute and one of the oldest universities in Europe.
„Mitochondria in Aging, Cancer and Cell Death” conference
On November 30th 2023 in Coimbra stared „Mitochondria in Aging, Cancer and Cell Death” international conference where I had the opportunity to give the talk entitled "Revealing of metabolic alterations in primary fibroblasts of patients with Neurodegeneration with Brain Iron Accumulation - subtype MPAN". Moreover, two postdocs from our MitoLab: Magdalena Lebiedzinska-Arciszewska and Patrycja Jakubek presented their recent work during the poster session.
I am very happy that I could meet my colleagues and friends in a such amazing scientific environment.
Visiting the LXV General Józef Bem High School
On November 22nd 2023 I have visited the LXV General Józef Bem High School with inclusion classes. My visit was aimed to familiarize students with the disease called: Neurodegeneration associated with iron deposition in the brain (NBIA). Moreover, I presented how scientists from the Nencki Institute are investigating metabolic disfunction in the fibroblasts derived from patients affected by this disease.
“What do we know about rare disease such as Neurodegeneration associated with iron deposition in the brain (NBIA). From bench to patient.” M.R. Wieckowski
My visit ended with a meeting with Małgorzata Pucułek, the director of the Gen. J. Bem High School and dr. Maciej Cwyl, the President of the NBIA Poland Association.
Our MitoLab team at the Free Radicals in Biology, Medicine, Sport and Nutrition conference in Gdańsk
Just yesterday we were at the NBIA conference in Warsaw and today our team from the MitoLab: prof. Mariusz Wieckowski, doctoral students (Agata Wydrych, Barbara Pakukła) and postdocs (dr Magdalena Lebiedzińska-Arciszewska and dr Patrycja Jakubek), presented the results of our latest research on metabolic disorders in neurodegenerative diseases and results on therapeutic possibilities based on stimulation of fatty acid metabolism, activation of autophagy and support for antioxidant protection in NAFLD and obesity.
Free Radicals in Biology, Medicine, Sport and Nutrition conference gathered many specialists in the field of mitochondria and metabolism.
First NBIA International Conference in Poland
"Scientists for the NBIA community"
From 19 to 21 October 2023 we participated in an extremely important event for NBIA community. This was the first International NBIA Conference entitled "Scientists for the NBIA community" organized in Poland. Several eminent scientists from different countries presented their study on NBIA (Neurodegeneration with Brain Iron Accumulation). It was great to meet all these wonderful people involved in helping patients and their families by investigating pathogenesis of this disease.
Official visit at the Jagiellonian University in Cracow
The September 14th had a place a working meeting at the Chair of Exercise Physiology and Muscle Bioenergetics, Faculty of Health Sciences, Jagiellonian University Medical College. Background of the photo is not accidental.
Working group: prof. Joanna Majerczak, prof. Jerzy Żołądź, prof. Agnieszka Dobrzyń, prof. Mariusz Więckowski and prof. Grzegorz Sumara.
UE Horizon RIA grant for our Laboratory
PAS GRAS scientific consortium, whose only Polish member is the Nencki Institute, is the winner of the European Commission's "Staying healthy" competition under the HORIZON-Research and Innovation Actions (RIA) program.
The coordinator of PasGras activities in Poland is Prof. Mariusz Więckowski from the MitoLab.
Research at the Nencki Institute will be carried out by 3 research teams led by: Prof. Mariusz Więckowski (Laboratory of Mitochondrial Biology and Metabolism), Prof. Agnieszka Dobrzyn (Laboratory of Cell Signaling and Metabolic Disorders) and Dr. Grzegorz Sumara (Laboratory of Metabolic Diseases).
The project is focused on de-risking metabolic, environmental and behavioral determinants of obesity in children, adolescents and young adults. The PAS GRAS consortium will be centered on overweight/obese children, adolescents and young adults with the main objective of reversing obesity and preventing later-life comorbidities. It will be determined how the lifestyle (diet, sedentary behaviours and physical activity), mental health, social/family factors, environment, interact with each person's intrinsic psychological, genetic and metabolic characteristics. PAS GRAS will also fill critical gaps in obesity diagnosis and prognosis and food/nutrition literacy, as well as will provide a blueprint for a healthier population.
The unique multi- and interdisciplinary PAS GRAS consortium led by Prof. Paulo Oliviera from University of Coimbra consists of 11 European Universities and Research Institutions as well as non-Academic partners: European Society for Clinical Investigation (Holand); Portuguese Diabetes Association (APDP); civil association AGDCentro Gymnastics Association (Portugal) and biomedical company Mediagnost GmbH (Germany).
PAS GRAS is one of the 6 projects funded under UE Horizon RIA Program, budget of the PAS GRAS project – 9,5 mln EUR.
Research collaboration with PACS2 Research Foundation
It is my great pleasure to announce that we've entered research collaboration with the PACS2 Research Foundation, which unites patients affected by ultra-rare genetic disease caused by autosomal dominant mutation in PACS2 gene that leads to developmental and epileptic encephalopathy (DEEs).
At the Nencki Institute the study will be conducted by our MitoLab team as well as by the team from the Laboratory of Cell Signalling and Metabolic Disorders headed by Prof. Agnieszka Dobrzyn.
A coordinated by me this collaborative research will contribute to a better understanding of the pathomechanism of this disease by filling the knowledge gaps regarding the impact of mutation in PACS2 gene on cellular metabolism.
Second bilateral Conference of the Nencki Institute and the Institute of Physiology of the Academy of Sciences of the Czech Republic
On October 11, the 2nd Conference of the Nencki Institute and the Institute of Physiology of the Academy of Sciences of the Czech Republic (IPHYS) was held at the Nencki Institute, coordinated by Prof. Mariusz Wieckowski and Prof. Adam Szewczyk from the Nencki Institute.
The topics of the conference focused on metabolism and neurobiology. The meeting aimed to establish new contacts between group leaders and create a platform for further collaboration. The conference included 14 presentations by participants from both institutes. Researchers from the Czech Republic also had the opportunity to visit laboratories at the Nencki Institute.
Grant COST
for
Prof. Mariusz Wieckowski
The consortium whose member is prof. Mariusz Wieckowski from the Laboratory of Mitochondrial Biology and Metabolism is among the winners of the COST action - acronym PRESTO „P2X receptors as a therapeutic opportunity”.
Prof. Wieckowski was appointed by Minister of Education and Science to represent Poland in the “PRESTO” COST Programme. The project is led by dr. Elena Adinolfi from the University of Ferrara. Consortium includes members from 20 different COST countries and two international partners from Brazil and Tunisia.
PRESTO Action aims not only at better understanding of the role of P2XRs in the pathogenesis of selected diseases, but also setting criteria for the validation of P2XRs as diagnostic and prognostic biomarkers and in principles accelerating the transition of P2XRs knowledge to clinical applications.
Grant OPUS-22 LAP from NCN
for Prof. Mariusz Wieckowski
The consortium led by Prof. Mariusz Wieckowski has been granted with OPUS-22 + LAP NCN funding, carried out in international collaboration under the WEAVE Programme for the project: „The role of impaired autophagy and peroxisome function in NAFLD development and their targeted recovery for improved efficacy of n-3 fatty acids”.
The consortium consists of Nencki Institute, Warsaw Medical University and Institute of Physiology of the Czech Academy of Sciences.
Science for Society
a new grant for our Laboratory
On March 23, 2022, by decision of the Minister of Education and Science, the Nencki Institute received a grant under the program "Science for Society" in the area - Scientific Excellence.
Project title: "Effect of omega 3,6,9 polyunsaturated fatty acids on metabolic profile of patients with neurodegeneration associated with iron deposition in the brain". The project leader is Prof. Mariusz R. Więckowski. Total budget is 1.9 million PLN.
The project will be implemented in cooperation with teams led by Prof. Mariusz Wieckowski and Prof. Agnieszka Dobrzyn from the Nencki Institute, and Prof. Iwona Kurkowska-Jastrzebska and dr hab. Marta Skowronska from the Institute of Psychiatry and Neurology in Warsaw. On the social side, the project is supported by the NBIA Poland Association, which unites NBIA patients and their families.
Ines Simoes received
PhD degree
It is with great satisfaction we announce that Ines Simoes (ESR 1) from our laboratory was proclaimed Doctor in Biological Sciences.
The objectives of her PhD, "Effects of Western diet in the development and progression of Non-alcoholic fatty liver disease", were to investigate the importance of mitochondria and peroxisomes in the development of NAFLD.
Congratulations to Dr. Inês Simoes for achieving this incredible milestone, and we wish you the best for the next chapter of your professional life!
Prof. Mariusz Wieckowski, the head of the Laboratory of Mitochondrial Biology and Metabolism at Nencki Institute, has been elected the councilor of the European Society for Clinical Investigation (ESCI) for the time frame of 2022-2024 starting officially in May 2022. The ESCI Council consists of 9-10 European councilors, chosen during and by the Annual Business Meeting of the Society.
The European Society for Clinical Investigation was established in 1967 as a pan-European Society to integrate clinicians and fundamental scientists with a genuine interest in uncovering the mechanisms involved in the pathophysiology of human disease. The objectives of the Society are the advancement of medical practice through science; the cultivation of clinical research by the methods of the natural sciences; the correlation of science with the art of medical practice; the fostering of high standards of ethical practice and investigation and the diffusion of a spirit of fraternity and international cooperation among and through its members. Society provides also a forum for discussion, debate and meeting colleagues in order to advance the science of clinical investigation. The term ‘clinical investigation’ is interpreted widely from bench to bedside including animal studies relevant to humans in health or disease.
Collaboration Agreement WITH NBIA Association Poland signed
Recently we've entered research collaboration with NBIA Association Poland, which unites patients affected by a rare genetic disease characterized by excessive brain iron accumulation (NBIA - neurodegeneration with brain iron accumulation).
The research that will be conducted in our Laboratory (in a close collaboration with the Laboratory of Cell Signalling and Metabolic Disorders headed by Prof. Agnieszka Dobrzyn also from Nencki Institute) will contribute to a better understanding of the pathomechanisms of NBIA disease most likely related to the alterations in lipid metabolism and mitochondrial dysfunction.
The tiger head present in the NBIA Poland Association's logo shows the characteristic -
"Tiger's Eye" visible in the MRI image of children diagnosed with this disease (especially the NBIA-PKAN type).
Towards a better understanding of Leigh Syndrome pathology and intervention published in Brain
A comprehensive review, co-authored by Mariusz R Wieckowski, on how Ndufs4 knockout mouse models have been used to unravel the pathomechanism of Leigh Syndrome has been published in the top-level journal „Brain“. This work is a collaborative effort between the Radboudumc, Wageningen University, The Nencki Institute of Experimental Biology (Warsaw), the Amsterdam UMC and the Universitat Autònoma de Barcelona (Spain).
Want to know more about this paper? Click on the link below for more news.
Collaboration Agreement between Nencki Institute and THERESON S.r.l. signed!
It is a great pleasure to announce that we've entered a research collaboration with THERESON a company focusing on therapeutic magnetic resonance.
Our Laboratory will support THERESON in the effort to expand the spectrum of applications of TMR® Therapeutic Magnetic Resonance, beyond wound care management.
THERESON is an Italian, medical device company, dedicated to the treatment of Vascular pathologies, related to Microcirculation, Orthopedic and Degenerative diseases. Their first medical device DIAPASON®, utilising TMR® Therapeutic Magnetic Resonance in wound care management, was launched to the market in 2017. The company is dedicated to clinical and scientific research and aims to demonstrate the effectiveness of the treatment through rigorously gathered experimental data.
