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Titolo:
Selective changes in mitochondria respiratory properties in oxidative or glycolytic muscle fibers isolated from G93AhumanSOD1 transgenic mice
Autore:
Leclerc, N; Ribera, F; Zoll, J; Warter, JM; Poindron, P; Lampert, E; Borg, J;
Indirizzi:
Univ Strasbourg 1, Fac Med, Lab Neurobiol Mol Interact Cellulaires, CNRS,ER 2072, F-67085 Strasbourg, France Univ Strasbourg 1 Strasbourg France F-67085 , F-67085 Strasbourg, France Univ Strasbourg 1, Ctr Rech Pharmaceut, Lab Pathol Commun Cellues Nerveuses & Musculaires, UPRES 2308, F-67401 Illkirch Graffenstaden, France Univ Strasbourg 1 Illkirch Graffenstaden France F-67401 enstaden, France Hop Univ, Serv Physiol Clin & Explorat Fonct, Unite Pluricent Etud Biol Muscle, F-67090 Strasbourg, France Hop Univ Strasbourg France F-67090 ol Muscle, F-67090 Strasbourg, France Hop Univ, Neurol Clin, F-67090 Strasbourg, France Hop Univ Strasbourg France F-67090 urol Clin, F-67090 Strasbourg, France
Titolo Testata:
NEUROMUSCULAR DISORDERS
fascicolo: 8, volume: 11, anno: 2001,
pagine: 722 - 727
SICI:
0960-8966(200111)11:8<722:SCIMRP>2.0.ZU;2-M
Fonte:
ISI
Lingua:
ENG
Soggetto:
AMYOTROPHIC-LATERAL-SCLEROSIS; CU,ZN-SUPEROXIDE DISMUTASE MUTANT; CU/ZN SUPEROXIDE-DISMUTASE; KINASE-DEFICIENT MICE; CREATINE-KINASE; MOTOR-NEURONS; HYDROGEN-PEROXIDE; SKELETAL-MUSCLE; IMPAIRMENT; DAMAGE;
Keywords:
amyotrophic lateral sclerosis; transgenic mouse; muscle function; oxidative stress; mitochondria; superoxide dismutase;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
35
Recensione:
Indirizzi per estratti:
Indirizzo: Borg, J Univ Strasbourg 1, Fac Med, Lab Neurobiol Mol Interact Cellulaires, CNRS,ER 2072, 11 Rue Humann, F-67085 Strasbourg, France Univ Strasbourg 111 Rue Humann Strasbourg France F-67085 France
Citazione:
N. Leclerc et al., "Selective changes in mitochondria respiratory properties in oxidative or glycolytic muscle fibers isolated from G93AhumanSOD1 transgenic mice", NEUROMUSC D, 11(8), 2001, pp. 722-727

Abstract

Cases of familial amyotrophic lateral sclerosis (FALS) are associated withmutations in cytosolic copper, zinc superoxide dismutase (SOD1). Total SODactivity and functional mitochondrial properties were studied in muscles and nervous tissues of control and transgenic mice mimicking the disease. Itwas found that total SOD activity was lower in nervous tissues than in muscles in both transgenic and control mice. In addition SOD activity increased during progression of disease in muscle but not in nervous tissue of transgenic mice. Maximal oxygen consumption and apparent Km for ADP were decreased in mitochondria from transgenic soleus (an oxidative muscle). However there was no difference between control and transgenic mice in respiratory parameters of mitochondria in the EDL muscle (a glycolytic muscle). These findings indicate that oxidative stress due to SOD1 mutations could alter energy metabolism in FALS mice. thereby affecting primarily oxidative muscle of the limbs, independently of motoneuron loss. (C) 2001 Elsevier Science B.V. All rights reserved.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 05/04/20 alle ore 23:00:30