Catalogo Articoli (Spogli Riviste)

OPAC HELP

Titolo:
Protective effects of cardiotrophin-1 adenoviral gene transfer on neuromuscular degeneration in transgenic ALS mice
Autore:
Bordet, T; Lesbordes, JC; Rouhani, S; Castelnau-Ptakhine, L; Schmalbruch, H; Haase, G; Kahn, A;
Indirizzi:
Inst Cochin Genet Mol, Dept Genet, F-75014 Paris, France Inst Cochin GenetMol Paris France F-75014 Genet, F-75014 Paris, France Inst Biol Dev Marseille, INSERM U382, F-13288 Marseille, France Inst Biol Dev Marseille Marseille France F-13288 13288 Marseille, France Univ Copenhagen, Panum Inst, Inst Med Physiol, DK-2200 Copenhagen, DenmarkUniv Copenhagen Copenhagen Denmark DK-2200 , DK-2200 Copenhagen, Denmark Hop Cochin, Serv Explorat Fonct, F-75014 Paris, France Hop Cochin Paris France F-75014 rv Explorat Fonct, F-75014 Paris, France
Titolo Testata:
HUMAN MOLECULAR GENETICS
fascicolo: 18, volume: 10, anno: 2001,
pagine: 1925 - 1933
SICI:
0964-6906(20010901)10:18<1925:PEOCAG>2.0.ZU;2-A
Fonte:
ISI
Lingua:
ENG
Soggetto:
AMYOTROPHIC-LATERAL-SCLEROSIS; PROGRESSIVE MOTOR NEURONOPATHY; CILIARY NEUROTROPHIC FACTOR; LEUKEMIA INHIBITORY FACTOR; SUPEROXIDE-DISMUTASE GENE; MOUSE MODEL; ANIMAL-MODEL; AXONAL-TRANSPORT; SELECTIVE LOSS; DISEASE;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
47
Recensione:
Indirizzi per estratti:
Indirizzo: Kahn, A Inst Cochin Genet Mol, Dept Genet, 24 Rue Fg St Jacques, F-75014 Paris, France Inst Cochin Genet Mol 24 Rue Fg St Jacques Paris France F-75014 e
Citazione:
T. Bordet et al., "Protective effects of cardiotrophin-1 adenoviral gene transfer on neuromuscular degeneration in transgenic ALS mice", HUM MOL GEN, 10(18), 2001, pp. 1925-1933

Abstract

Amyotrophic lateral sclerosis (ALS) is mainly a sporadic neurodegenerativedisorder characterized by loss of cortical and spinal motoneurons. Some familial ALS cases (FALS) have been linked to dominant mutations in the gene encoding Cu/Zn superoxide dismutase (SOD1). Transgenic mice overexpressing a mutated form of human SOD1 with a Gly(93)Ala substitution develop progressive muscle wasting and paralysis as a result of spinal motoneuron loss anddie at 5 to 6 months. We investigated the effects of neurotrophic factor gene delivery in this FALS model. Intramuscular injection of an adenoviral vector encoding cardiotrophin-1 (CT-1) in SOD1(G93A) newborn mice resulted in systemic delivery of CT-1, supplying motoneurons with a continuous sourceof trophic factor. CT-1 delayed the onset of motor impairment as assessed in the rotarod test. Axonal degeneration was slowed and skeletal muscle atrophy was largely reduced by CT-1 treatment. By monitoring the amplitude of the evoked motor response, we showed that the time-course of motor impairment was significantly decreased by CT-1 treatment. Thus, adenovirus-mediatedgene transfer of neurotrophic factors might delay neurogenic muscular atrophy and progressive neuromuscular deficiency in ALS patients.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 29/03/20 alle ore 23:16:58