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Titolo:
MUSCLE CARNITINE DEFICIENCY AND LIPID STORAGE MYOPATHY IN PATIENTS WITH MITOCHONDRIAL MYOPATHY
Autore:
CAMPOS Y; HUERTAS R; BAUTISTA J; GUTIERREZ E; APARICIO M; LORENZO G; SEGURA D; VILLANUEVA M; CABELLO A; ALESSO L; ARENAS J;
Indirizzi:
HOSP 12 OCTUBRE,CTR INVEST,AVE CORDOBA KM 52 E-28041 MADRID SPAIN HOSP 12 OCTUBRE,CTR INVEST,AVE CORDOBA KM 52 E-28041 MADRID SPAIN HOSP VIRGEN ROCIO,SERV NEUROL SEVILLE SPAIN HOSP RAMON & CAJAL,SERV NEUROPEDIAT MADRID SPAIN DEPT MED,SIGMA TAU LAB MADRID SPAIN
Titolo Testata:
Muscle & nerve
fascicolo: 7, volume: 16, anno: 1993,
pagine: 778 - 781
SICI:
0148-639X(1993)16:7<778:MCDALS>2.0.ZU;2-J
Fonte:
ISI
Lingua:
ENG
Soggetto:
C-OXIDASE DEFICIENCY; HUMAN SKELETAL-MUSCLE; METABOLISM;
Keywords:
CARNITINE DEFICIENCY; MITOCHONDRIA; LIPID ACCUMULATION; RAGGED-RED FIBER; RESPIRATORY CHAIN;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
18
Recensione:
Indirizzi per estratti:
Citazione:
Y. Campos et al., "MUSCLE CARNITINE DEFICIENCY AND LIPID STORAGE MYOPATHY IN PATIENTS WITH MITOCHONDRIAL MYOPATHY", Muscle & nerve, 16(7), 1993, pp. 778-781

Abstract

Abnormal carnitine distribution in muscle was found in 22 of 77 patients (29%), with mitochondrial myopathy. Furthermore, total (TC) and free (FC) carnitine levels in muscle were lower in patients than in controls (P < 0.01). Muscle long-chain acylcarnitines (LCAC) were significantly increased in these patients (P < 0.01). Muscle carnitine deficiency was found in 31.5% of patients with lipid storage myopathy (LSM) and in 25.6% of patients with ragged-red fibers (RRF). Therefore, carnitine deficiency can be found in patients with mitochondrial myopathy even in the absence of LSM. Muscle levels of TC and FC were lower in patients with respiratory chain defects than in those with normal respiratory chain (P < 0.01). In contrast, LCAC levels were significantly increased (P < 0.05). Carnitine levels did not differ significantly, among patients with different respiratory-chain defects. Consequently, these patients, owing to their biochemical block, reduce progressively the muscle carnitine pool and subsequent LCAC rise, due to long-chain fatty acid (LCFA) accumulation.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 10/07/20 alle ore 09:44:10