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Audioslide presentation on Claudia Manzoni's paper examining how fibroblasts with LRRK2 mutations react to starvation conditions and the possible deficits that they have in autophagy.

LRRK2 and autophagy in fibroblasts

In this paper Claudia Manzoni studies how fibroblast cells from people with Parkinson’s disease caused by mutations in LRRK2 react to starvation. Although the changes are quite subtle, there are differences between the way that fibroblasts that contain mutant LRRK2 respond to being starved – suggesting that there may be changes in the way that these cells regulate a key process called autophagy (a term which comes from the greek meaning to eat yourself, and is one of the ways that cells get rid of waste and recycle proteins and organellles).
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Drosophila fly model - University of Sheffield

Genetic mutations linked to Parkinson's disease

Research led by consortium researchers Dr Helene Plun-Favreau (UCL Institute of Neurology) and Dr Alex Whitworth (University of Sheffield), and collaborator Dr Heike Laman (University of Cambridge), has discovered how genetic mutations linked to Parkinson’s disease might play a key role in the death of brain cells, potentially paving the way for the development of more effective drug treatments. In the new study, published in Nature Neuroscience, the team of cross-institutional researchers showed how defects in the Parkinson’s gene Fbxo7 cause problems with mitophagy. More...

Autophagy

LRRK2 and autophagy

Mutations in LRRK2 are the most common genetic cause of Parkinson’s disease. Here, Claudia Manzoni talks about her research (funded by the Rosetrees Trust and the Michael J. Fox Foundation) into what LRRK2 might be doing within the cell: Parkinson’s disease is a brain illness that afflicts 1 in 500 people in the UK. High profile patients, such as the actor Michael J Fox, the boxer Muhammad Ali and the late Pope John Paul II, have raised public awareness of Parkinson’s and its devastating impact. More...

GBA neurons

GBA and mitochondria

Dr Laura Osellame tells us about her recent paper in Cell Metabolism about Mitochondrial dysfunction linked to loss of an enzyme called GBA: Gaucher Disease (GD) is a rare inherited disease, belonging to the family of lysosomal storage disorders. Mutations in the gene glucocerebrosidase (GBA) are responsible for the disease and can increase susceptibility to Parkinson’s disease (PD). Genetic studies undertaken at UCL and other hospitals around the world suggest that mutations in GBA are the most common genetic risk factor currently known for PD. More...

Image of alpha-synuclein

Alpha-synuclein in LRRK2 brains

First author Adamantios Mamais tells us about his recent publication in Neurobiology of Disease: At the Queen Square Brain Bank (part of the UCL Institute of Neurology) we hold a large collection of post-mortem human brain tissue from patients with neurodegenerative diseases including Parkinson’s disease (PD); a debilitating neurological disorder that affects the central nervous system. In the United States alone about 50,000 new cases are reported every year. The main symptoms include tremor, slow movement, rigid limbs and a shuffling gait while these worsen with time. More...

Helene Plun-Favreau

(MRC Research Fellow)


My scientific career has always followed my interest in signalling pathways in normal as well as in pathological conditions. During the three years of my PhD work at INSERM U564 in Angers, France, I received specialist training on cellular biology and biochemistry. My graduate work allowed me to identify a new cytokine in the Interleukin-6 family responsible for motor neuron survival. I then focused on the structure of the cytokines of this family, which allowed me to be acquainted with modelling studies, as well as on the specific signalling pathways influenced by these molecules. I graduated in December 2002 and then decided to pursue my career in a signal transduction laboratory. Being aware of both Cancer Research UK and Julian Downward’s renown, I joined his laboratory in 2002 as a post-doctoral fellow. During that period, I have been led to work on apoptosis and Parkinson’s Disease (PD). I studied the regulation of HtrA2 by PINK1, two mitochondrial proteins implicated in PD.

My ambitions were to pursue academic scientific-clinical research in the PD field. Having been awarded a career development award in September 2007, I am currently leading the Neurological Signaling Group in the Molecular Neuroscience department of the Institute of Neurology, UCL. The major interest of my group is to dissect the molecular pathways implicated in PD

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Jump to: 2014 | 2013 | 2012 | 2011 | 2010 | 2009 | 2008 | 2007 | 2005 | 2004 | 2003 | 2002 | 2001 | 2000
Number of items: 33.

2014

Birsa, N; Norkett, R; Wauer, T; Mevissen, TE; Wu, HC; Foltynie, T; ... Kittler, JT; + view all (2014) K27 ubiquitination of the mitochondrial transport protein Miro is dependent on serine 65 of the Parkin ubiquitin ligase. J Biol Chem 10.1074/jbc.M114.563031.

Tufi, R; Gandhi, S; de Castro, IP; Lehmann, S; Angelova, PR; Dinsdale, D; ... Martins, LM; + view all (2014) Enhancing nucleotide metabolism protects against mitochondrial dysfunction and neurodegeneration in a PINK1 model of Parkinson's disease. Nat Cell Biol 10.1038/ncb2901.

2013

Bartolome, F; Wu, HC; Burchell, VS; Preza, E; Wray, S; Mahoney, CJ; ... Plun-Favreau, H; + view all (2013) Pathogenic VCP mutations induce mitochondrial uncoupling and reduced ATP levels. Neuron , 78 (1) 57 - 64. 10.1016/j.neuron.2013.02.028.

Burchell, VS; Nelson, DE; Sanchez-Martinez, A; Delgado-Camprubi, M; Ivatt, RM; Pogson, JH; ... Plun-Favreau, H; + view all (2013) The Parkinson's disease-linked proteins Fbxo7 and Parkin interact to mediate mitophagy. Nat Neurosci 10.1038/nn.3489.

Manzoni, C; Mamais, A; Dihanich, S; Abeti, R; Soutar, MP; Plun-Favreau, H; ... Lewis, PA; + view all (2013) Inhibition of LRRK2 kinase activity stimulates macroautophagy. Biochim Biophys Acta 10.1016/j.bbamcr.2013.07.020.

Manzoni, C; Mamais, A; Dihanich, S; McGoldrick, P; Devine, MJ; Zerle, J; ... Lewis, PA; + view all (2013) Pathogenic Parkinson's disease mutations across the functional domains of LRRK2 alter the autophagic/lysosomal response to starvation. Biochem Biophys Res Commun 10.1016/j.bbrc.2013.10.159.

2012

Fitzgerald, JC; Camprubi, MD; Dunn, L; Wu, HC; Ip, NY; Kruger, R; ... Plun-Favreau, H; + view all (2012) Phosphorylation of HtrA2 by cyclin-dependent kinase-5 is important for mitochondrial function. Cell Death Differ , 19 (2) 257 - 266. 10.1038/cdd.2011.90.

Plun-Favreau, H; Burchell, VS; Holmström, KM; Yao, Z; Deas, E; Cain, K; ... Abramov, AY; + view all (2012) HtrA2 deficiency causes mitochondrial uncoupling through the F₁F₀-ATP synthase and consequent ATP depletion. Cell Death and Disease , 3 , Article e335. 10.1038/cddis.2012.77. Green and gold open access
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2011

Deas, E; Plun-Favreau, H; Gandhi, S; Desmond, H; Kjaer, S; Loh, SHY; ... Wood, NW; + view all (2011) PINK1 cleavage at position A103 by the mitochondrial protease PARL. Human Molecular Genetics , 20 (5) pp. 867-879. 10.1093/hmg/ddq526. Green open access
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Deas, E; Wood, NW; Plun-Favreau, H; (2011) Mitophagy and Parkinson's disease: The PINK1-parkin link. BBA-MOL CELL RES , 1813 (4) 623 - 633. 10.1016/j.bbamcr.2010.08.007.

Devine, MJ; Plun-Favreau, H; Wood, NW; (2011) Parkinson's disease and cancer: two wars, one front. NAT REV CANCER , 11 (11) 812 - +. 10.1038/nrc3150.

Yao, Z; Gandhi, S; Burchell, VS; Plun-Favreau, H; Wood, NW; Abramov, AY; (2011) Cell metabolism affects selective vulnerability in PINK1-associated Parkinson's disease. J Cell Sci , 124 (Pt 24) 4194 - 4202. 10.1242/jcs.088260. Gold open access

2010

Burchell, VS; Gandhi, S; Deas, E; Wood, NW; Abramov, AY; Plun-Favreau, H; (2010) Targeting mitochondrial dysfunction in neurodegenerative disease: Part II. EXPERT OPIN THER TAR , 14 (5) 497 - 511. 10.1517/14728221003730434.

Burchell, VS; Gandhi, S; Deas, E; Wood, NW; Abramov, AY; Plun-Favreau, H; (2010) Targeting mitochondrial dysfunction in neurodegenerative disease: Part I. EXPERT OPIN THER TAR , 14 (4) 369 - 385. 10.1517/14728221003652489.

Plun-Favreau, H; Lewis, PA; Hardy, J; Martins, LM; Wood, NW; (2010) Cancer and Neurodegeneration: Between the Devil and the Deep Blue Sea. PLOS GENET , 6 (12) , Article e1001257. 10.1371/journal.pgen.1001257. Gold open access

Yao, Z; Gandhi, S; Plun-Favreau, H; Wood, NW; Abramov, AY; (2010) PINK1 Deficiency and Mitochondrial Dysfunction in Neurons and Skeletal Myocytes. BIOPHYSICAL JOURNAL , 98 (3) 381A - 381A.

2009

Deas, E; Plun-Favreau, H; Wood, NW; (2009) PINK1 function in health and disease. EMBO MOL MED , 1 (3) 152 - 165. 10.1002/emmm.200900024. Gold open access

Gandhi, S; Wood-Kaczmar, A; Yao, Z; Plun-Favreau, H; Deas, E; Klupsch, K; ... Abramov, AY; + view all (2009) PINK1-Associated Parkinson's Disease Is Caused by Neuronal Vulnerability to Calcium-induced Cell Death. MOL CELL , 33 (5) 627 - 638. 10.1016/j.molcel.2009.02.013. Gold open access

Moisoi, N; Klupsch, K; Fedele, V; East, P; Sharma, S; Renton, A; ... Martins, LM; + view all (2009) Mitochondrial dysfunction triggered by loss of HtrA2 results in the activation of a brain-specific transcriptional stress response. CELL DEATH DIFFER , 16 (3) 449 - 464. 10.1038/cdd.2008.166.

Tain, LS; Chowdhury, RB; Tao, RN; Plun-Favreau, H; Moisoi, N; Martins, LM; ... Tapon, N; + view all (2009) Drosophila HtrA2 is dispensable for apoptosis but acts downstream of PINK1 independently from Parkin. CELL DEATH DIFFER , 16 (8) 1118 - 1125. 10.1038/cdd.2009.23.

2008

Fitzgerald, JC; Plun-Favreau, H; (2008) Emerging pathways in genetic Parkinson's disease: Autosomal-recessive genes in Parkinson's disease - a common pathway? FEBS J , 275 (23) 5758 - 5766. 10.1111/j.1742-4658.2008.06708.x.

Plun-Favreau, H; Gandhi, S; Wood-Kaczmar, A; Deas, E; Yao, Z; Wood, NW; (2008) What Have PINK1 and HtrA2 Genes Told Us about the Role of Mitochondria in Parkinson's Disease? MITOCHONDRIA AND OXIDATIVE STRESS IN NEURODEGENERATIVE DISORDERS , 1147 30 - 36. 10.1196/annals.1427.032.

Plun-Favreau, H; Hardy, J; (2008) PINK1 in mitochondrial function. P NATL ACAD SCI USA , 105 (32) 11041 - 11042. 10.1073/pnas.0805908105. Gold open access

2007

Plun-Favreau, H; Klupsch, K; Moisoi, N; Gandhi, S; Kjaer, S; Frith, D; ... Downward, J; + view all (2007) The mitochondrial protease HtrA2 is regulated by Parkinson's disease-associated kinase PINK1. NAT CELL BIOL , 9 (11) 1243 - U63. 10.1038/ncb1644.

2005

Strauss, KM; Martins, LM; Plun-Favreau, H; Marx, FP; Kautzmann, S; Berg, D; ... Krüger, R; + view all (2005) Loss of function mutations in the gene encoding Omi/HtrA2 in Parkinson's disease. Human Molecular Genetics , 15 (14) 2099 - 2111.

Strauss, KM; Martins, LM; Plun-Favreau, H; Marx, FP; Kautzmann, S; Berg, D; ... Kruger, R; + view all (2005) Loss of function mutations in the gene encoding Omi/HtrA2 in Parkinson's disease. HUM MOL GENET , 14 (15) 2099 - 2111. 10.1093/hmg/ddi215.

2004

Perret, D; Guillet, C; Elson, G; Froger, J; Plun-Favreau, H; Rousseau, F; ... Gascan, H; + view all (2004) Two different contact sites are recruited by cardiotrophin-like cytokine (CLC) to generate the CLC/CLF and CLC/sCNTFR alpha composite cytokines. J BIOL CHEM , 279 (42) 43961 - 43970. 10.1074/jbc.M407686200. Gold open access

2003

Plun-Favreau, H; Perret, D; Diveu, C; Froger, J; Chevalier, S; Lelievre, E; ... Chabbert, M; + view all (2003) Leukemia inhibitory factor (LIF), cardiotrophin-1, and oncostatin M share structural binding determinants in the immunoglobulin-like domain of LIF receptor. J BIOL CHEM , 278 (29) 27169 - 27179. 10.1074/jbc.M303168200. Gold open access

2002

Guillet, C; Lelievre, E; Plun-Favreau, H; Froger, J; Chabbert, M; Hermann, J; ... Elson, G; + view all (2002) Functionally active fusion protein of the novel composite cytokine CLC/soluble CNTF receptor. EUR J BIOCHEM , 269 (7) 1932 - 1941. 10.1046/j.1432-1033.2002.02850.x.

Lisbonne, M; Plun-Favreau, H; (2002) French Society of Immunology: Cytokine Meeting May 16-17, 2002, Le Croisic, Port-aux-Rocs, France. Eur Cytokine Netw , 13 (4) 474 - 476.

2001

Lelievre, E; Plun-Favreau, H; Chevalier, S; Froger, J; Guillet, C; Elson, GCA; ... Gascan, H; + view all (2001) Signaling pathways recruited by the cardiotrophin-like cytokine/cytokine-like factor-1 composite cytokine - Specific requirement of the membrane-bound form of ciliary neurotrophic factor receptor alpha component. J BIOL CHEM , 276 (25) 22476 - 22484. Gold open access

Plun-Favreau, H; Elson, G; Chabbert, M; Froger, J; deLapeyriere, O; Lelievre, E; ... Chevalier, S; + view all (2001) The ciliary neurotrophic factor receptor alpha component induces the secretion of and is required for functional responses to cardiotrophin-like cytokine. EMBO J , 20 (7) 1692 - 1703.

2000

Elson, GCA; Lelievre, E; Guillet, C; Chevalier, S; Plun-Favreau, H; Froger, J; ... Gascan, H; + view all (2000) CLF associates with CLC to form a functional heteromeric ligand for the CNTF receptor complex. NAT NEUROSCI , 3 (9) 867 - 872.

This list was generated on Fri Apr 18 00:00:45 2014 BST.

Page last modified on 20 mar 13 17:01