Regulation of the Mitochondrial Proteome

 

Description

Our research focuses on mitochondrial biogenesis, in particular the transcriptional regulation of nuclear encoded mitochondrial genes. Our aim is to understand how mitochondrial biogenesis mediates adaptation to cellular stress under pathological conditions, including neurodegenerative disease, myopathies and and cancer. To this aim we apply live cell imaging, molecular biology and bioinformatic approaches on cellular and animal models.

5 recent publications

  • Logan CV*, Szabadkai G*, Sharpe JA*, Parry DA, Torelli S, Childs A-M, Kriek M, Phadke R, Johnson CA, Roberts NY, Bonthron DT, Pysden KA, Whyte T, Munteanu I, Foley AR, Wheway G, Szymanska K, Natarajan S, Abdelhamed ZA, Morgan JE, Roper H, Santen GWE, Niks EH, van der Pol WL, Lindhout D, Raffaello A, De Stefani D, den Dunnen JT, Sun Y, Ginjaar I, et al.: Loss-of-function mutations in MICU1 cause a brain and muscle disorder linked to primary alterations in mitochondrial calcium signaling. Nat Genet 2014, 46:188–93.
  • Blacker TS, Mann ZF, Gale JE, Ziegler M, Bain AJ, Szabadkai G*, Duchen MR*: Separating NADH and NADPH fluorescence in live cells and tissues using FLIM. Nat Commun 2014, 5(May):3936.
  • Yao Z, Jones a WE, Fassone E, Sweeney MG, Lebiedzinska M, Suski JM, Wieckowski MR, Tajeddine N, Hargreaves IP, Yasukawa T, Tufo G, Brenner C, Kroemer G, Rahman S, Szabadkai G: PGC-1β mediates adaptive chemoresistance associated with mitochondrial DNA mutations. Oncogene 2013, 32:2592–600.
  • Astin R, Bentham R, Djafarzadeh S, Horscroft J a, Kuc RE, Leung PS, Skipworth JR a, Vicencio JM, Davenport AP, Murray AJ, Takala J, Jakob SM, Montgomery H, Szabadkai G: No evidence for a local renin-angiotensin system in liver mitochondria. Sci Rep 2013, 3:2467.
  • Botta A, Malena A, Loro E, Del Moro G, Suman M, Pantic B, Szabadkai G, Vergani L: Altered Ca2+ homeostasis and endoplasmic reticulum stress in myotonic dystrophy type 1 muscle cells. Genes  2013, 4:275–92.

 

Grants

  • AIRC
  • Telethon