Literature DB >> 19741215

Analysis of FUS gene mutation in familial amyotrophic lateral sclerosis within an Italian cohort.

N Ticozzi1, V Silani, A L LeClerc, P Keagle, C Gellera, A Ratti, F Taroni, T J Kwiatkowski, D M McKenna-Yasek, P C Sapp, R H Brown, J E Landers.   

Abstract

OBJECTIVE: Mutations in the FUS gene on chromosome 16 have been recently discovered as a cause of familial amyotrophic lateral sclerosis (FALS). This study determined the frequency and identities of FUS gene mutations in a cohort of Italian patients with FALS.
METHODS: We screened all 15 coding exons of FUS for mutations in 94 Italian patients with FALS.
RESULTS: We identified 4 distinct missense mutations in 5 patients; 2 were novel. The mutations were not present in 376 healthy Italian controls and thus are likely to be pathogenic.
CONCLUSIONS: Our results demonstrate that FUS mutations cause approximately 4% of familial amyotrophic lateral sclerosis cases in the Italian population.

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Year:  2009        PMID: 19741215      PMCID: PMC2764725          DOI: 10.1212/WNL.0b013e3181bbff05

Source DB:  PubMed          Journal:  Neurology        ISSN: 0028-3878            Impact factor:   9.910


  39 in total

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2.  Identification and characterization of the nuclear localization/retention signal in the EWS proto-oncoprotein.

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3.  Incidence of ALS in Lombardy, Italy.

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Journal:  Neurology       Date:  2007-01-09       Impact factor: 9.910

4.  An increased specificity score matrix for the prediction of SF2/ASF-specific exonic splicing enhancers.

Authors:  Philip J Smith; Chaolin Zhang; Jinhua Wang; Shern L Chew; Michael Q Zhang; Adrian R Krainer
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Review 5.  Molecular biology of amyotrophic lateral sclerosis: insights from genetics.

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7.  Focality of upper and lower motor neuron degeneration at the clinical onset of ALS.

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8.  Two Italian kindreds with familial amyotrophic lateral sclerosis due to FUS mutation.

Authors:  Adriano Chiò; Gabriella Restagno; Maura Brunetti; Irene Ossola; Andrea Calvo; Gabriele Mora; Mario Sabatelli; Maria Rosaria Monsurrò; Stefania Battistini; Jessica Mandrioli; Fabrizio Salvi; Rossella Spataro; Jennifer Schymick; Bryan J Traynor; Vincenzo La Bella
Journal:  Neurobiol Aging       Date:  2009-05-17       Impact factor: 4.673

9.  Identification of new ANG gene mutations in a large cohort of Italian patients with amyotrophic lateral sclerosis.

Authors:  Cinzia Gellera; Claudia Colombrita; Nicola Ticozzi; Barbara Castellotti; Cinzia Bragato; Antonia Ratti; Franco Taroni; Vincenzo Silani
Journal:  Neurogenetics       Date:  2007-12-18       Impact factor: 2.660

10.  SNAP: predict effect of non-synonymous polymorphisms on function.

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  54 in total

1.  De novo truncating FUS gene mutation as a cause of sporadic amyotrophic lateral sclerosis.

Authors:  Mariely DeJesus-Hernandez; Jannet Kocerha; NiCole Finch; Richard Crook; Matt Baker; Pamela Desaro; Amelia Johnston; Nicola Rutherford; Aleksandra Wojtas; Kathleen Kennelly; Zbigniew K Wszolek; Neill Graff-Radford; Kevin Boylan; Rosa Rademakers
Journal:  Hum Mutat       Date:  2010-05       Impact factor: 4.878

Review 2.  RNA processing pathways in amyotrophic lateral sclerosis.

Authors:  Marka van Blitterswijk; John E Landers
Journal:  Neurogenetics       Date:  2010-03-27       Impact factor: 2.660

3.  Combined FDG and raclopride PET study in a case of ALS with the R521C FUS gene mutation.

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Journal:  J Neurol       Date:  2011-07-15       Impact factor: 4.849

Review 4.  Misregulated RNA processing in amyotrophic lateral sclerosis.

Authors:  Magdalini Polymenidou; Clotilde Lagier-Tourenne; Kasey R Hutt; C Frank Bennett; Don W Cleveland; Gene W Yeo
Journal:  Brain Res       Date:  2012-03-03       Impact factor: 3.252

Review 5.  Biological Spectrum of Amyotrophic Lateral Sclerosis Prions.

Authors:  Magdalini Polymenidou; Don W Cleveland
Journal:  Cold Spring Harb Perspect Med       Date:  2017-11-01       Impact factor: 6.915

Review 6.  Clinical genetics of amyotrophic lateral sclerosis: what do we really know?

Authors:  Peter M Andersen; Ammar Al-Chalabi
Journal:  Nat Rev Neurol       Date:  2011-10-11       Impact factor: 42.937

7.  A de novo missense mutation of the FUS gene in a "true" sporadic ALS case.

Authors:  Adriano Chiò; Andrea Calvo; Cristina Moglia; Irene Ossola; Maura Brunetti; Luca Sbaiz; Shiao-lin Lai; Yevgeniya Abramzon; Bryan J Traynor; Gabriella Restagno
Journal:  Neurobiol Aging       Date:  2010-07-03       Impact factor: 4.673

8.  Intranuclear aggregation of mutant FUS/TLS as a molecular pathomechanism of amyotrophic lateral sclerosis.

Authors:  Takao Nomura; Shoji Watanabe; Kumi Kaneko; Koji Yamanaka; Nobuyuki Nukina; Yoshiaki Furukawa
Journal:  J Biol Chem       Date:  2013-11-26       Impact factor: 5.157

Review 9.  TDP-43/FUS in motor neuron disease: Complexity and challenges.

Authors:  Erika N Guerrero; Haibo Wang; Joy Mitra; Pavana M Hegde; Sara E Stowell; Nicole F Liachko; Brian C Kraemer; Ralph M Garruto; K S Rao; Muralidhar L Hegde
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10.  Nomenclature and nosology for neuropathologic subtypes of frontotemporal lobar degeneration: an update.

Authors:  Ian R A Mackenzie; Manuela Neumann; Eileen H Bigio; Nigel J Cairns; Irina Alafuzoff; Jillian Kril; Gabor G Kovacs; Bernardino Ghetti; Glenda Halliday; Ida E Holm; Paul G Ince; Wouter Kamphorst; Tamas Revesz; Annemieke J M Rozemuller; Samir Kumar-Singh; Haruhiko Akiyama; Atik Baborie; Salvatore Spina; Dennis W Dickson; John Q Trojanowski; David M A Mann
Journal:  Acta Neuropathol       Date:  2009-11-19       Impact factor: 17.088

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