Dr Anne Bertolotti

Anne Bertolotti

University position

Programme leader

Dr Anne Bertolotti is pleased to consider applications from prospective PhD students.




Medical Research Council Laboratory of Molecular Biology

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Research Theme

Cellular and Molecular Neuroscience


Cellular aspects of protein misfolding in neurodegenerative diseases

Neurodegenerative diseases characterized by accumulation of misfolded proteins are late-onset, implying that the proteins responsible for these diseases cause cellular dysfunction specifically in aged neurones while being benign for many years. Using various complementary approaches, our aim is to characterize the events triggering pathological protein aggregation. Our work might contribute to a better comprehension of the molecular mechanisms allowing the cells to cope with the burden of misfolded proteins. In addition, we may open the way to a better understanding of the aging process.

Research Focus







Clinical conditions

Amyotrophic lateral sclerosis

Huntington's disease


Molecular and cell biology


No collaborators listed



Münch C, Bertolotti A (2011), “Self-propagation and transmission of misfolded mutant SOD1: Prion or prion-like phenomenon?” Cell Cycle 10(11):1711 Details

Münch C, O'Brien J, Bertolotti A (2011), “Prion-like propagation of mutant superoxide dismutase-1 misfolding in neuronal cells.” Proc Natl Acad Sci U S A 108(9):3548-53 Details

Tsaytler P, Harding HP, Ron D, Bertolotti A (2011), “Selective Inhibition of a Regulatory Subunit of Protein Phosphatase 1 Restores Proteostasis.” Science Details


Münch C, Bertolotti A (2010), “Exposure of hydrophobic surfaces initiates aggregation of diverse ALS-causing superoxide dismutase-1 mutants.” J Mol Biol 399(3):512-25 Details


Rousseau E, Kojima R, Hoffner G, Djian P, Bertolotti A (2009), “Misfolding of proteins with a polyglutamine expansion is facilitated by proteasomal chaperones.” J Biol Chem 284(3):1917-29 Details


Dehay B, Weber C, Trottier Y, Bertolotti A (2007), “Mapping of the epitope of monoclonal antibody 2B4 to the proline-rich region of human Huntingtin, a region critical for aggregation and toxicity.” Biotechnol J 2(5):559-64 Details


Dehay B, Bertolotti A (2006), “Critical role of the proline-rich region in Huntingtin for aggregation and cytotoxicity in yeast.” J Biol Chem 281(47):35608-15 Details


Rousseau E, Dehay B, Ben-Haïem L, Trottier Y, Morange M, Bertolotti A (2004), “Targeting expression of expanded polyglutamine proteins to the endoplasmic reticulum or mitochondria prevents their aggregation.” Proc Natl Acad Sci U S A 101(26):9648-53 Details


Marcu MG, Doyle M, Bertolotti A, Ron D, Hendershot L, Neckers L (2002), “Heat shock protein 90 modulates the unfolded protein response by stabilizing IRE1alpha.” Mol Cell Biol 22(24):8506-13 Details


Bertolotti A, Ron D (2001), “Alterations in an IRE1-RNA complex in the mammalian unfolded protein response.” J Cell Sci 114(Pt 17):3207-12 Details

Bertolotti A, Wang X, Novoa I, Jungreis R, Schlessinger K, Cho JH, West AB, Ron D (2001), “Increased sensitivity to dextran sodium sulfate colitis in IRE1beta-deficient mice.” J Clin Invest 107(5):585-93 Details


Bertolotti A, Zhang Y, Hendershot LM, Harding HP, Ron D (2000), “Dynamic interaction of BiP and ER stress transducers in the unfolded-protein response.” Nat Cell Biol 2(6):326-32 Details

Harding HP, Zhang Y, Bertolotti A, Zeng H, Ron D (2000), “Perk is essential for translational regulation and cell survival during the unfolded protein response.” Mol Cell 5(5):897-904 Details

Urano F, Bertolotti A, Ron D (2000), “IRE1 and efferent signaling from the endoplasmic reticulum.” J Cell Sci 113 Pt 21:3697-702 Details

Urano F, Wang X, Bertolotti A, Zhang Y, Chung P, Harding HP, Ron D (2000), “Coupling of stress in the ER to activation of JNK protein kinases by transmembrane protein kinase IRE1.” Science 287(5453):664-6 Details


Bertolotti A, Bell B, Tora L (1999), “The N-terminal domain of human TAFII68 displays transactivation and oncogenic properties.” Oncogene 18(56):8000-10 Details


Bertolotti A, Melot T, Acker J, Vigneron M, Delattre O, Tora L (1998), “EWS, but not EWS-FLI-1, is associated with both TFIID and RNA polymerase II: interactions between two members of the TET family, EWS and hTAFII68, and subunits of TFIID and RNA polymerase II complexes.” Mol Cell Biol 18(3):1489-97 Details


Bertolotti A, Lutz Y, Heard DJ, Chambon P, Tora L (1996), “hTAF(II)68, a novel RNA/ssDNA-binding protein with homology to the pro-oncoproteins TLS/FUS and EWS is associated with both TFIID and RNA polymerase II.” EMBO J 15(18):5022-31 Details