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Nuoffer Jean-Marc

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BEC 2020.1 Mitochondrial physiology
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COST Action CA15203 (2016-2021): MitoEAGLE
Evolution-Age-Gender-Lifestyle-Environment: mitochondrial fitness mapping

Nuoffer Jean-Marc

MitoPedia topics: EAGLE 


Name Nuoffer Jean-Marc, Prof. Dr.
Institution Universitätsinstitut für klinische Chemie, Inselspital, INO F
Address , 3010
City Bern
Country Switzerland
Email [email protected]
O2k-Network Lab CH Bern Nuoffer JM



Deng 2022 Cell Mol Life Sci2022Deng H, Gao Y, Trappetti V, Hertig D, Karatkevich D, Losmanova T, Urzi C, Ge H, Geest GA, Bruggmann R, Djonov V, Nuoffer JM, Vermathen P, Zamboni N, Riether C, Ochsenbein A, Peng RW, Kocher GJ, Schmid RA, Dorn P, Marti TM (2022) Targeting lactate dehydrogenase B-dependent mitochondrial metabolism affects tumor initiating cells and inhibits tumorigenesis of non-small cell lung cancer by inducing mtDNA damage.
Melin 2022 JHEP Rep2022Melin N, Yarahmadov T, Sanchez-Taltavull D, Birrer FE, Brodie TM, Petit B, Felser A, Nuoffer JM, Montani M, Vozenin MC, Herrmann E, Candinas D, Aebersold DM, Stroka D (2022) A new mouse model of radiation-induced liver disease reveals mitochondrial dysfunction as an underlying fibrotic stimulus.
Gschwind 2022 Ann Clin Transl Neurol2022Gschwind M, Garcia Segarra N, Schaller A, Bolognini R, Nuoffer JM, Hourez R, Deprez M, Lhermitte B, Maeder P, Tran C, Kuntzer T (2022) Early-onset leukoencephalomyelopathy due to a biallelic NDUFV1 variant in a mid-forties patient.
BEC 2020.1 doi10.26124bec2020-0001.v12020Gnaiger E et al ― MitoEAGLE Task Group (2020) Mitochondrial physiology.
Rajasekaran 2018 FASEB J2018Rajasekaran R, Felser A, Nuoffer JM, Dufour JF, St-Pierre MV (2018) The histidine triad nucleotide-binding protein 2 (HINT-2) positively regulates hepatocellular energy metabolism. FASEB J 32:5143-61.
Mueller 2017 Orphanet J Rare Dis2017Mueller SM, Gehrig SM, Petersen JA, Frese S, Mihaylova V, Ligon-Auer M, Khmara N, Nuoffer JM, Schaller A, Lundby C, Toigo M, Jung HH (2017) Effects of endurance training on skeletal muscle mitochondrial function in Huntington disease patients. Orphanet J Rare Dis 12:184.
Jackson 2014 J Med Genet2014Jackson CB, Nuoffer JM, Hahn D, Prokisch H, Haberberger B, Gautsch M, Häberli A, Gallati S, Schaller A (2014) Mutations in SDHD lead to autosomal recessive encephalomyopathy and isolated mitochondrial Complex II deficiency. J Med Genet 51:170-5.
Hirsch 2012 PloS One2012Hirsch A, Hahn D, Kempná P, Hofer G, Mullis PE, Nuoffer JM, Flück CE (2012) Role of AMP-activated protein kinase on steroid hormone biosynthesis in adrenal NCI-H295R cells. PLoS One 7:e30956.


Eggimann 2014 Abstract MiP20142014Adaptation to nutrient availability in human fibroblasts with mitochondrial dysfunction: the role of sirtuins.
Hahn 2014 Abstract MiP20142014Validation of oxygen consumption measurements in muscle and fibroblasts from patients with mitochondrial diseases.


Thank you very much for your commitment to this project. My group has experience especially in the diagnosis of mitochondrial diseases from muscle biopsies or cultures of fibroblasts. In addition, we have measured cell lines (COS, HepG, or adrenal cells) during the cause of some of our projects. Standardization of protocols but also pre-analytics are, in my opinion, very important topics. To gain insight into the comparability of the different methods, it would, furthermore, be very important to have the option of an external quality control (e.g. as interlaboratory tests), as done by ERNDIM for different analyses. I would be happy to contribute. - Jean-Marc Nuoffer 2016. Translated by Georg Kandolf
        • Benefits for COST and for the COST Action MitoEAGLE
My group mainly has experience in the diagnosis of mitochondriaopathies from muscle biopsies or fibroblast cultures. However, we have already measured cell lines (COS, HepG, or adrenal cortex cell lines) in individual research projects. The standardization of the protocols as well as the paeanalytic is, in my opinion, an extremely important topic. In order to gain insight into the comparability of the different methods, it would also be very important to have an external quality control (eg as a ring test) as is the case with ERNDIM for other analyzes.