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Titolo:
DEFECTIVE BRAIN ENERGY-METABOLISM SHOWN BY INVIVO P-31 MR SPECTROSCOPY IN 28 PATIENTS WITH MITOCHONDRIAL CYTOPATHIES
Autore:
BARBIROLI B; MONTAGNA P; MARTINELLI P; LODI R; IOTTI S; CORTELLI P; FUNICELLO R; ZANIOL P;
Indirizzi:
UNIV BOLOGNA,IST CLIN NEUROL,VIA U FOSCOLO 7 I-40123 BOLOGNA ITALY UNIV BOLOGNA,CATTEDRA BIOCHIM CLIN I-40123 BOLOGNA ITALY UNIV MODENA,IST RADIOL I-41100 MODENA ITALY
Titolo Testata:
Journal of cerebral blood flow and metabolism
fascicolo: 3, volume: 13, anno: 1993,
pagine: 469 - 474
SICI:
0271-678X(1993)13:3<469:DBESBI>2.0.ZU;2-5
Fonte:
ISI
Lingua:
ENG
Soggetto:
MAGNETIC-RESONANCE SPECTROSCOPY; ENCEPHALOMYOPATHY MERRF; OPHTHALMOPLEGIA-PLUS; OXIDATIVE-METABOLISM; RABBIT BRAIN; MUSCLE; CEREBELLAR; MYOPATHY; DISEASE; FAMILY;
Keywords:
BRAIN BIOENERGETICS; IMPAIRED BRAIN ENERGY METABOLISM; MITOCHONDRIAL CYTOPATHIES; BRAIN MR SPECTROSCOPY;
Tipo documento:
Article
Natura:
Periodico
Citazioni:
34
Recensione:
Indirizzi per estratti:
Citazione:
B. Barbiroli et al., "DEFECTIVE BRAIN ENERGY-METABOLISM SHOWN BY INVIVO P-31 MR SPECTROSCOPY IN 28 PATIENTS WITH MITOCHONDRIAL CYTOPATHIES", Journal of cerebral blood flow and metabolism, 13(3), 1993, pp. 469-474

Abstract

We studied brain energy metabolism by phosphorus magnetic resonance spectroscopy (P-31 MRS) in 28 patients with mitochondrial cytopathies, and 20 normal control subjects. Fourteen patients had myopathy alone, six had only mild brain symptoms, and eight showed different degrees of brain involvement. Brain P-31 MRS showed a low phosphocreatine content in all patients, accompanied by a high inorganic phosphate in 14 of28 patients. The average value of the P(i) concentration in the patient group was significantly (p = 0.009) different from the control group. The cytosolic pH was normal. From these data were derived a high concentration of ADP (calculated from the creatine kinase equilibrium), a high percent value of V/V(max) for ATP biosynthesis, and a low phosphorylation potential, all features showing a derangement of brain energy metabolism, in all patients with mitochondrial cytopathies. P-31 MRS proved to be sensitive enough to disclose a deficit of mitochondrialfunctionality not only in the affected patients, but also in those without clinically evident brain symptoms.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 19/01/20 alle ore 07:03:58