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Titolo:
Mitochondrial encephalomyopathies: therapeutic approaches
Autore:
DiMauro, S; Hirano, M; Schon, EA;
Indirizzi:
Columbia Univ Coll Phys & Surg, Dept Neurol, New York, NY 10032 USA Columbia Univ Coll Phys & Surg New York NY USA 10032 w York, NY 10032 USA Columbia Univ Coll Phys & Surg, Dept Genet & Dev, New York, NY 10032 USA Columbia Univ Coll Phys & Surg New York NY USA 10032 w York, NY 10032 USA
Titolo Testata:
NEUROLOGICAL SCIENCES
fascicolo: 5, volume: 21, anno: 2000, supplemento:, S
pagine: S901 - S908
SICI:
1590-1874(2000)21:5<S901:META>2.0.ZU;2-0
Fonte:
ISI
Lingua:
ENG
Soggetto:
KEARNS-SAYRE-SYNDROME; SYSTEMIC CARNITINE DEFICIENCY; COENZYME Q(10) DEFICIENCY; RESPIRATORY-CHAIN; LACTIC-ACIDOSIS; SKELETAL-MUSCLE; COMPLEX-III; IN-VITRO; NEUROGASTROINTESTINAL ENCEPHALOMYOPATHY; OXIDATIVE-PHOSPHORYLATION;
Keywords:
mitochondrial encephalomyopathies; therapy;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Citazioni:
75
Recensione:
Indirizzi per estratti:
Indirizzo: DiMauro, S Columbia Univ Coll Phys & Surg, Dept Neurol, 630 W 168th St, New York, NY 10032 USA Columbia Univ Coll Phys & Surg 630 W 168th St New YorkNY USA 10032
Citazione:
S. DiMauro et al., "Mitochondrial encephalomyopathies: therapeutic approaches", NEUROL SCI, 21(5), 2000, pp. S901-S908

Abstract

Therapy of mitochondrial encephalomyopathies (defined restrictively as defects of the mitochondrial respiratory chain) is woefully inadequate, despite great progress in our understanding of the molecular bases of these disorders. We review available and experimental therapeutic approaches, which fall into seven categories: (1) palliative therapy; (2) removal of noxious metabolites; (3) administration of artificial electron accepters; (4) administration of metabolites and cofactors; (5) administration of oxygen radical scavengers; (6) gene therapy; and (7) genetic counseling. Progress in each of these approaches provides some glimmer of hope for the future, although much work remains to be done.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 30/03/20 alle ore 13:22:00