Dr Anne Bertolotti

Anne Bertolotti

University position

Programme leader

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

Departments

MRC LMB

Institutes

Medical Research Council Laboratory of Molecular Biology

Home page

http://www2.mrc-lmb.cam.ac.uk/group-l...

Research Theme

Cellular and Molecular Neuroscience

Interests

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

Keywords

Folding

amyloid

Chaperones

aggregation

Huntington

Clinical conditions

Amyotrophic lateral sclerosis

Huntington's disease

Equipment

Molecular and cell biology

Collaborators

No collaborators listed

Publications

2011

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

2010

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

2009

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

2007

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

2006

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

2004

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

2002

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

2001

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

2000

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

1999

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

1998

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

1996

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