Amyotrophic lateral sclerosis

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder that is marked by fasciculation, spasticity and progressive weakness of muscles, and results in difficulty speaking, swallowing and breathing. ALS is fatal, usually leading to death within a few years from diagnosis, although more slowly progressing forms of the disease exist.

Latest Research and Reviews

  • Research | | open

    • Robert Kueffner
    • , Neta Zach
    • , Maya Bronfeld
    • , Raquel Norel
    • , Nazem Atassi
    • , Venkat Balagurusamy
    • , Barbara Di Camillo
    • , Adriano Chio
    • , Merit Cudkowicz
    • , Donna Dillenberger
    • , Javier Garcia-Garcia
    • , Orla Hardiman
    • , Bruce Hoff
    • , Joshua Knight
    • , Melanie L. Leitner
    • , Guang Li
    • , Lara Mangravite
    • , Thea Norman
    • , Liuxia Wang
    • , Rached Alkallas
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    • , Jeanne Avril
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    • , Yoav Bar-Sinai
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    • , Bhavna Ahuja Nicole Corriveau
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    • , Fan Fan
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    • , Devin C. Koestler
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    • , Huan-Jui Chang
    •  & Gustavo Stolovitzky
  • Research |

    Klim et al. illuminate pathomechanisms of ALS using pluripotent stem cells to identify transcripts altered in human motor neurons by perturbations to ALS protein TDP-43, finding the microtubule regulator STMN2 highly sensitive to TDP-43 malfunctions.

    • Joseph R. Klim
    • , Luis A. Williams
    • , Francesco Limone
    • , Irune Guerra San Juan
    • , Brandi N. Davis-Dusenbery
    • , Daniel A. Mordes
    • , Aaron Burberry
    • , Michael J. Steinbaugh
    • , Kanchana K. Gamage
    • , Rory Kirchner
    • , Rob Moccia
    • , Seth H. Cassel
    • , Kuchuan Chen
    • , Brian J. Wainger
    • , Clifford J. Woolf
    •  & Kevin Eggan
    Nature Neuroscience 22, 167-179
  • Research |

    The mRNA encoding stathmin-2, a protein implicated in axonal growth, is shown to be widely suppressed by premature polyadenylation in both sporadic and C9orf72 ALS through a mechanism directly dependent on loss of nuclear TDP-43 in motor neurons.

    • Ze’ev Melamed
    • , Jone López-Erauskin
    • , Michael W. Baughn
    • , Ouyang Zhang
    • , Kevin Drenner
    • , Ying Sun
    • , Fernande Freyermuth
    • , Moira A. McMahon
    • , Melinda S. Beccari
    • , Jon W. Artates
    • , Takuya Ohkubo
    • , Maria Rodriguez
    • , Nianwei Lin
    • , Dongmei Wu
    • , C. Frank Bennett
    • , Frank Rigo
    • , Sandrine Da Cruz
    • , John Ravits
    • , Clotilde Lagier-Tourenne
    •  & Don W. Cleveland
    Nature Neuroscience 22, 180-190
  • Protocols |

    This protocol describes how to inject therapeutic cells, viruses, or other macromolecular products into the mouse or rat lumbar ventral cord, modeling the routes used for administration of therapies currently in use in human clinical trials.

    • Kevin S. Chen
    • , Lisa M. McGinley
    • , Osama N. Kashlan
    • , John M. Hayes
    • , Elizabeth S. Bruno
    • , Josh S. Chang
    • , Faye E. Mendelson
    • , Maegan A. Tabbey
    • , Karl Johe
    • , Stacey A. Sakowski
    •  & Eva L. Feldman
    Nature Protocols 14, 331-349
  • Research |

    Using a newly developed biochemical method for aggregated protein extraction, Laferrière and colleagues uncover different neurotoxic types of pathologic TDP-43 assemblies in the brains of subjects with distinct subtypes of frontotemporal dementia.

    • Florent Laferrière
    • , Zuzanna Maniecka
    • , Manuela Pérez-Berlanga
    • , Marian Hruska-Plochan
    • , Larissa Gilhespy
    • , Eva-Maria Hock
    • , Ulrich Wagner
    • , Tariq Afroz
    • , Paul J. Boersema
    • , Gery Barmettler
    • , Sandrine C. Foti
    • , Yasmine T. Asi
    • , Adrian M. Isaacs
    • , Ashraf Al-Amoudi
    • , Amanda Lewis
    • , Henning Stahlberg
    • , John Ravits
    • , Francesca De Giorgi
    • , François Ichas
    • , Erwan Bezard
    • , Paola Picotti
    • , Tammaryn Lashley
    •  & Magdalini Polymenidou

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