Motor neuron degeneration, severe myopathy and TDP-43 increase in a transgenic pig model of SOD1-linked familiar ALS.

Amyotrophic Lateral Sclerosis / genetics Animals Animals, Genetically Modified Disease Models, Animal Humans Motor Neurons / pathology Muscular Diseases / genetics Nerve Degeneration / genetics Superoxide Dismutase-1 / genetics Swine TDP-43 Proteinopathies / genetics
ALS Amyotrophic lateral sclerosis SOD1 TDP-43 Transgenic pig

Journal

Neurobiology of disease
ISSN: 1095-953X
Titre abrégé: Neurobiol Dis
Pays: United States
ID NLM: 9500169

Informations de publication

Date de publication:
04 2019
Historique:
received: 20 03 2018
revised: 26 10 2018
accepted: 19 11 2018
pubmed: 25 11 2018
medline: 30 11 2019
entrez: 25 11 2018
Statut: ppublish

Résumé

Amyotrophic Lateral Sclerosis (ALS) is a neural disorder gradually leading to paralysis of the whole body. Alterations in superoxide dismutase SOD1 gene have been linked with several variants of familial ALS. Here, we investigated a transgenic (Tg) cloned swine model expressing the human pathological hSOD1

Identifiants

DOI: 10.1016/j.nbd.2018.11.021 PMID: 30471417
pubmed: 30471417
pii: S0969-9961(18)30752-6
doi: 10.1016/j.nbd.2018.11.021
pii:
doi:

Substances chimiques

SOD1 protein, human 0
Superoxide Dismutase-1 EC 1.15.1.1

Types de publication

Journal Article Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

263-275

Informations de copyright

Copyright © 2018 The Authors. Published by Elsevier Inc. All rights reserved.

Auteurs

Paola Crociara (P)

Istituto Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d'Aosta, Via Bologna 148, 10154 Turin, TO, Italy.

Maria Novella Chieppa (MN)

Istituto Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d'Aosta, Via Bologna 148, 10154 Turin, TO, Italy.

Elena Vallino Costassa (E)

Istituto Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d'Aosta, Via Bologna 148, 10154 Turin, TO, Italy.

Elena Berrone (E)

Istituto Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d'Aosta, Via Bologna 148, 10154 Turin, TO, Italy.

Marina Gallo (M)

Istituto Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d'Aosta, Via Bologna 148, 10154 Turin, TO, Italy.

Monica Lo Faro (M)

Istituto Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d'Aosta, Via Bologna 148, 10154 Turin, TO, Italy.

Maria Domenica Pintore (MD)

Istituto Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d'Aosta, Via Bologna 148, 10154 Turin, TO, Italy.

Barbara Iulini (B)

Istituto Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d'Aosta, Via Bologna 148, 10154 Turin, TO, Italy.

Antonio D'Angelo (A)

Department of Veterinary Sciences, University of Turin, Largo Paolo Braccini 2, 10095 Grugliasco, TO, Italy.

Giovanni Perona (G)

Department of Veterinary Sciences, University of Turin, Largo Paolo Braccini 2, 10095 Grugliasco, TO, Italy.

Alberto Botter (A)

Laboratorio di Ingegneria del Sistema Neuromuscolare (LISIN), Politecnico di Torino, Corso Castelfidardo 42/a, 10138 Turin, TO, Italy.

Donato Formicola (D)

NeuroMuscularFunction research group, School of Exercise and Sport Sciences, Department of Medical Sciences, University of Turin, Piazza Gian Lorenzo Bernini, 12, 10143 Turin, TO, Italy.

Alberto Rainoldi (A)

NeuroMuscularFunction research group, School of Exercise and Sport Sciences, Department of Medical Sciences, University of Turin, Piazza Gian Lorenzo Bernini, 12, 10143 Turin, TO, Italy.

Marianna Paulis (M)

Milan Unit, Istituto di Ricerca Genetica e Biomedica, CNR, Via Fantoli 16/15, 20138 Milan, MI, Italy; Humanitas Clinical and Research Center, via Manzoni 56, 20089 Rozzano, MI, Italy.

Paolo Vezzoni (P)

Milan Unit, Istituto di Ricerca Genetica e Biomedica, CNR, Via Fantoli 16/15, 20138 Milan, MI, Italy; Humanitas Clinical and Research Center, via Manzoni 56, 20089 Rozzano, MI, Italy.

Federica Meli (F)

Istituto di Genetica Molecolare, CNR, Via Abbiategrasso 207, 27100 Pavia, PV, Italy.

Fiorenzo Antonio Peverali (FA)

Istituto di Genetica Molecolare, CNR, Via Abbiategrasso 207, 27100 Pavia, PV, Italy.

Caterina Bendotti (C)

IRCCS-Istituto di Ricerche Farmacologiche "Mario Negri", Via La Masa, 19, 20156 Milano, MI, Italy.

Maria Chiara Trolese (MC)

IRCCS-Istituto di Ricerche Farmacologiche "Mario Negri", Via La Masa, 19, 20156 Milano, MI, Italy.

Laura Pasetto (L)

IRCCS-Istituto di Ricerche Farmacologiche "Mario Negri", Via La Masa, 19, 20156 Milano, MI, Italy.

Valentina Bonetto (V)

IRCCS-Istituto di Ricerche Farmacologiche "Mario Negri", Via La Masa, 19, 20156 Milano, MI, Italy.

Giovanna Lazzari (G)

Avantea, Via Porcellasco 7F, 26100 Cremona, CR, Italy; Fondazione Avantea, Via Porcellasco 7F, 26100 Cremona, CR, Italy.

Roberto Duchi (R)

Avantea, Via Porcellasco 7F, 26100 Cremona, CR, Italy.

Andrea Perota (A)

Avantea, Via Porcellasco 7F, 26100 Cremona, CR, Italy.

Irina Lagutina (I)

Avantea, Via Porcellasco 7F, 26100 Cremona, CR, Italy.

Corinne Quadalti (C)

Avantea, Via Porcellasco 7F, 26100 Cremona, CR, Italy.

Maria Silvia Gennero (MS)

Istituto Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d'Aosta, Via Bologna 148, 10154 Turin, TO, Italy.

Daniela Dezzutto (D)

Istituto Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d'Aosta, Via Bologna 148, 10154 Turin, TO, Italy.

Rosanna Desiato (R)

Istituto Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d'Aosta, Via Bologna 148, 10154 Turin, TO, Italy.

Marina Boido (M)

Neuroscience Institute Cavalieri Ottolenghi (NICO), University of Turin, Department of Neuroscience, Regione Gonzole 10, 10043 Orbassano, TO, Italy.

Matilde Ghibaudi (M)

Neuroscience Institute Cavalieri Ottolenghi (NICO), University of Turin, Department of Neuroscience, Regione Gonzole 10, 10043 Orbassano, TO, Italy.

Maria Consuelo Valentini (MC)

Department of Neuroradiology, A.O.U. Città della Salute e della Scienza, Corso Bramante 88, 10126 Turin, TO, Italy.

Maria Caramelli (M)

Istituto Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d'Aosta, Via Bologna 148, 10154 Turin, TO, Italy.

Cesare Galli (C)

Avantea, Via Porcellasco 7F, 26100 Cremona, CR, Italy; Fondazione Avantea, Via Porcellasco 7F, 26100 Cremona, CR, Italy.

Cristina Casalone (C)

Istituto Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d'Aosta, Via Bologna 148, 10154 Turin, TO, Italy.

Cristiano Corona (C)

Istituto Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d'Aosta, Via Bologna 148, 10154 Turin, TO, Italy. Electronic address: cristiano.corona@izsto.it.

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Classifications MeSH

questionsmedicales.fr › Maladies métaboliques et nutritionnelles › Maladies métaboliques › Troubles de l'homéostasie des protéines › Protéinopathies TDP-43 › Sclérose latérale amyotrophique › Motor neuron degeneration, severe myopathy and...
questionsmedicales.fr › Eucaryotes › Animaux › Motor neuron degeneration, severe myopathy and...
questionsmedicales.fr › Stades d'organismes › Organismes génétiquement modifiés › Animal génétiquement modifié › Motor neuron degeneration, severe myopathy and...
questionsmedicales.fr › Techniques d'investigation › Modèles théoriques › Modèles biologiques › Modèles animaux de maladie humaine › Motor neuron degeneration, severe myopathy and...
questionsmedicales.fr › Eucaryotes › Animaux › Chordés › Vertébrés › Mammifères › Eutheria › Primates › Haplorhini › Catarrhini › Hominidae › Humains › Motor neuron degeneration, severe myopathy and...
questionsmedicales.fr › Cellules › Neurones › Neurones efférents › Motoneurones › Motor neuron degeneration, severe myopathy and...
questionsmedicales.fr › Maladies du système nerveux › Maladies neuromusculaires › Maladies musculaires › Motor neuron degeneration, severe myopathy and...
questionsmedicales.fr › États, signes et symptômes pathologiques › Processus pathologiques › Dégénérescence nerveuse › Motor neuron degeneration, severe myopathy and...
questionsmedicales.fr › Enzymes et coenzymes › Enzymes › Oxidoreductases › Superoxide dismutase › Superoxide dismutase-1 › Motor neuron degeneration, severe myopathy and...
questionsmedicales.fr › Eucaryotes › Animaux › Chordés › Vertébrés › Mammifères › Eutheria › Artiodactyla › Suidae › Motor neuron degeneration, severe myopathy and...
questionsmedicales.fr › Maladies métaboliques et nutritionnelles › Maladies métaboliques › Troubles de l'homéostasie des protéines › Protéinopathies TDP-43 › Motor neuron degeneration, severe myopathy and...