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Titolo:
ABNORMAL CALCIUM HOMEOSTASIS AND MITOCHONDRIAL POLARIZATION IN A HUMAN ENCEPHALOMYOPATHY
Autore:
MOUDY AM; HANDRAN SD; GOLDBERG MP; RUFFIN N; KARL I; KRANZEBLE P; DEVIVO DC; ROTHMAN SM;
Indirizzi:
ST LOUIS CHILDRENS HOSP,DEPT NEUROL,1 CHILDRENS PL ST LOUIS MO 63110 ST LOUIS CHILDRENS HOSP,DEPT NEUROL ST LOUIS MO 63110 WASHINGTON UNIV,SCH MED,CTR STUDY NERVOUS SYSTEM INJURY,DEPT NEUROL ST LOUIS MO 63110 WASHINGTON UNIV,SCH MED,DEPT MED,DIV ENDOCRINOL & METAB ST LOUIS MO 63110 COLUMBIA UNIV,COLL PHYS & SURG,NEUROL INST NEW YORK NEW YORK NY 10032
Titolo Testata:
Proceedings of the National Academy of Sciences of the United Statesof America
fascicolo: 3, volume: 92, anno: 1995,
pagine: 729 - 733
SICI:
0027-8424(1995)92:3<729:ACHAMP>2.0.ZU;2-4
Fonte:
ISI
Lingua:
ENG
Soggetto:
LACTIC-ACIDOSIS; MEMBRANE-POTENTIALS; CLINICAL-FEATURES; EPISODES MELAS; DNA; ENCEPHALOPATHY; MYOPATHY; MUTATION; DISEASE; CELLS;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
38
Recensione:
Indirizzi per estratti:
Citazione:
A.M. Moudy et al., "ABNORMAL CALCIUM HOMEOSTASIS AND MITOCHONDRIAL POLARIZATION IN A HUMAN ENCEPHALOMYOPATHY", Proceedings of the National Academy of Sciences of the United Statesof America, 92(3), 1995, pp. 729-733

Abstract

Patients with several inherited human encephalomyopathies exhibit systemic and neurological symptoms in association with specific mitochondrial mutations. The mechanisms by which these mitochondrial mutations result in cellular injury have not been elucidated. One potential cause of neuronal vulnerability is an inability to effectively buffer intracellular calcium. We report that fibroblasts from patients with one specific inherited encephalomyopathy, MELAS (mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes) syndrome, have elevated levels of ionized calcium and cannot normally sequester calcium influxes. Quantitative fluorescence imaging demonstrated that this abnormality was associated with a relative decrease in mitochondrial membrane potential compared to control fibroblasts. This documentation of pathological calcium homeostasis in a genetic neurological disease extends the calcium hypothesis of toxic cell injury to human mitochondrial encephalomyopathies.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 10/07/20 alle ore 19:06:31