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Cells Webinar | The Pathomechanism of Mitochondrial Diseases

11 Oct 2021, 16:00 (CEST)

Mitochondrial Disease, Pathomechanism, Mitochondrial Respiratory Chain, Mitochondrial Structure, Stress Response, Metabolic Adaptation
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Welcome from the Chairs

11th Webinar on Cells

The Pathomechanism of Mitochondrial Diseases

Mitochondria harbor many vital biochemical pathways and play crucial roles in cellular functions, including apoptosis and calcium hoeostasis. However, their primary task is to provide cellular energy via oxidative phosphorylation (OXPHOS). Mitochondrial diseases are a heterogenous group of common inborn errors of metabolism, originating from pathogenic variants in the mitochondrial or nuclear genome, resulting in OXPHOS deficiency, mostly with devastating consequences. Furthermore, mitochondrial dysfunction is also associated with numerous other pathological conditions, including neurodegenerative diseases, diabetes, cancer, immune dysfunction, and heart and kidney diseases.

This webinar features several eminent experts in mitochondrial biology, who study function and metabolic adaptation to stress and diseases in different model systems.

Date: 11 October 2021

Time: 4:00 pm CEST | 10:00 am EDT | 10:00 pm CST Asia

Webinar ID: 864 9851 7924

Webinar Secretariat: cells.webinar@mdpi.com

Chair

Department of Genetics Hadassah Medical Center and Faculty of Medicince the Hebrew University of Jerusalem, Israel

DETAILS
Bio
Dr. Ann Saada (Reisch) is interested in the pathomechanism of inborn errors of metabolism. After studying Microbiology (PhD Supervisor, Prof.Itzak Kahane) and Neurobiology (Post.Doc, Host Prof. Shlomo Rothshenker) at the Hebrew University of Jerusalem Israel, she joined the Metabolic Disease Unit, Shaare Zedek Medical Center in Jerusalem (headed by Prof Orly Elpeleg). There, she directed the Enzyme Diagnostics Laboratory. In 2005, the unit transferred to, and merged with, the Genetics Deparment at Hadassah Medical Center, where she managed the Enzyme Diagnostic Laboratory, and subsequently also the Metabolite Diagnostics Laboratory. In parallel with diagnostic services, she is an independent researcher, who joined the Faculty of Medicine, Hebrew University of Jerusalem, where she is now an Associate Professor. Her research group, which is funded by exernal research grants, (presently the Israel Science Foundation (ISF)), focuses on the pathomechanism of nuclear encoded, mitochondrial diseases, and in collaborations, the mitochondrial respiratory chain in neurodegenerative diseases and diabetes.

Invited Speakers

Saarland University School of Medicine, Department of Medical Biochemistry & Molecular Biology, Center for Molecular Signalling (PZMS), Germany

DETAILS
Bio
I studied Biology at the University of Osnabrück, Germany, where I completed my diploma degree at the Department of Microbiology under the supervision of Prof. Dr. Karlheinz Altendorf. As a predoctoral student, I moved to the Netherlands to the group of Prof. Dr. Arnold Driessen at Groningen University, Department of Molecular Microbiology, studying bacterial membrane protein biogenesis pathways, and obtained my PhD in 2004. Supported by an EMBO long-term fellowship, I then joined the team of Prof. Dr. Nikolaus Pfanner at the Department of Biochemistry and Molecular Biology of the University of Freiburg, Germany, as a postdoctoral researcher investigating the molecular mechanisms of mitochondrial protein import and membrane insertion. In 2007, I became an independent research group leader at the same department, deepening and broadening my great interest in the biogenesis and functional architecture of mitochondria. One of our major achievements during that time was the discovery of the membrane-shaping and -tethering Mitochondrial Contact Site and Cristae Organizing System (MICOS). Since 2015, I have been a full professor of Physiological Chemistry at the Saarland University Medical School. We are currently studying how different molecular machineries that control the intricate ultrastructure of mitochondrial membranes and/or form contact sites with other cellular membrane systems are coordinated in space and time, and how these complex systems are remodelled during metabolic adaptation and stress responses.

Shmunis School of Biomedicine & Cancer Research, George S. Wise Faculty of Life Sciences, Sagol School of Neuroscience, Tel Aviv University, Israel

DETAILS
Bio
Orna Elroy-Stein is a full professor at Tel Aviv University (Israel). She carried out her undergraduate education in Molecular Biology at the Technion, the Israel Institute of Technology. Interested in Human Molecular Genetics, she completed her PhD under the supervision of Prof. Dr. Yoram Groner at the Weismann Institute of Science in Israel, where she studies the molecular biology of Down's syndrome. During her post-doctoral studies with Dr. Bernard Moss at the Lab of Viral Diseases at NIH (Maryland, USA), she developed vaccinia virus-based expression vectors for mammalian systems. The Faculty of Life Sciences at Tel Aviv University recruited her in 1991 to establish her own research group. Over the years, she has been awarded multiple research grants from numerous sources, including the Israel Science Foundation (ISF), US-Israel Binational Foundation (BSF) and European Leukodystrophy Association (ELA). Her lab focuses on projects related to the regulation of mRNA translation in health and disease, employing a multi-technique approach, which includes quantitative 'omics' at the transcriptome, translatome and proteome levels. Her group focuses on two main projects: (i) regulation of protein synthesis during the cell cycle; and (ii) molecular mechanisms of vanishing white matter (VWM), a genetic neurodegenerative disease caused by mutations in the translation initiation factor eIF2B. Her group generated the first mouse model for VWM disease and found a connection to mitochondrial malfunction.

The Liver Unit, Hadassah-Hebrew University Medical Center, Jerusalem, Israel

DETAILS
Bio
Sarah Weksler-Zangen completed her PhD at the Hebrew University, Jerusalem, Israel. Following her PhD, she performed her postdoctoral studies in the molecular genetics and physiology of hypertension at Harvard University Hospital (MGH and BWH), with Prof. Howard Jacob and Prof. Hollenberg NK as mentors. Upon returning to Israel in 1996, Dr. Weksler-Zangen established her own laboratory, focusing on pancreatic β-cell failure in type 2 diabetes (T2D). Uniquely, she characterized and studied a genetic rat model of nutritionally induced T2D, the Cohen diabetic sensitive (CDs) rat. Using this model, she first considered the possibility that a primary defect in the mitochondrial respiration chain may be the cause of a selective impairment in the pancreatic β-cell function of these rats. She received the Melita Bern-Schlanger prize from the Hebrew University Center for these studies. The pathogenesis underlying β-cell failure in type 2 diabetes (T2D), the role of the dysfunctional mitochondria and the identification of interventional measures to prevent and protect the β-cells from the harmful environment have been the focus of her research over the years.

Program

Speaker/Presentation

Time in CEST

Chair Prof. Dr. Ann Saada

Chair Introduction

4:00 - 4:10 pm

Prof. Dr. Martin van der Laan

Biogenesis and Functional Architecture of Mitochondrial Membranes

4:10 - 4:40 pm

Prof. Dr. Orna Elroy-Stein

The Energy Status of Astrocytes Is the Achilles’ Heel of eIF2B-Leukodystrophy

4:40 - 5:10 pm

Dr. Sarah Weksler-Zangen

Is Type 2 diabetes a primary mitochondrial disorder?

5:10 - 5:40 pm

Q&A Session

5:40 - 5:55 pm

Closing of Webinar

Chair Prof. Dr. Ann Saada

5:55 - 6:00 pm

Webinar Content

On Monday, 11 October 2021, MDPI and the Journal Cells organized the 11th webinar on Cells, entitled "The Pathomechanism of Mitochondrial Diseases". Chaired by Prof. Dr. Ann Saada from the Hadassah Medical Center and the Hebrew University of Jerusalem, the webinar discussed mitochondrial function and its metabolic adaptation to stress and diseases.

The first speaker to kick off the webinar was Prof. Dr. Martin van der Laan, who discussed the biogenesis and functional architecture of mitochondrial membranes. Prof. Dr. Orna Elroy-Stein followed with her talk on the defective energy status of astrocytes in eIF2B-Leukodystrophy. Finally, Dr. Sarah Weksler-Zangen discussed if type 2 diabetes can be considered a primary mitochondrial disorder. Q&A sessions were conducted after each speaker to answer any questions submitted by live online audience and a short discussion was held at the end of the webinar.

The webinar was offered via Zoom, and registration was required for attendance. The full recording can be found below. To stay updated on upcoming Cells webinars organized by MDPI, be sure to sign up for our newsletter by clicking on “Subscribe” at the top of the page.

Relevant SI

Role of Mitochondria in Environmentally and Dietary Modulated Age-Associated Diseases
Guest Editors: Dr. Natascia Ventura and Prof. Dr. Judith Haendeler
Deadline for manuscript submissions:15 November 2021

The Pathomechanism of Mitochondrial Diseases
Guest Editor: Prof. Ann Saada
Deadline for manuscript submissions: 10 January 2022

Mitochondrial Functionality in Liver Pathologies
Guest Editor: Dr. Matilde Bustos
Deadline for manuscript submissions: 28 February 2022

Closed Special Issue

Mitochondria Meet Iron Metabolism: Facts and Opportunities
Guest Editors: Prof. Dr. Francesco Costanzo and Dr. Flavia Biamonte
Deadline for manuscript submissions: closed (30 June 2021)

Sponsors and Partners

Organizers

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