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Titolo:
CSF insulin-like growth factor-1 in infantile neuronal ceroid lipofuscinosis
Autore:
Riikonen, R; Vanhanen, SL; Tyynela, J; Santavuori, P; Turpeinen, U;
Indirizzi:
Kuopio Univ Hosp, Kuopio, Finland Kuopio Univ Hosp Kuopio FinlandKuopio Univ Hosp, Kuopio, Finland Hosp Children & Adolescents, Helsinki, Finland Hosp Children & Adolescents Helsinki Finland escents, Helsinki, Finland Univ Helsinki, Inst Biomed, FIN-00014 Helsinki, Finland Univ Helsinki Helsinki Finland FIN-00014 ed, FIN-00014 Helsinki, Finland Univ Helsinki, Cent Hosp Lab, FIN-00014 Helsinki, Finland Univ Helsinki Helsinki Finland FIN-00014 ab, FIN-00014 Helsinki, Finland
Titolo Testata:
NEUROLOGY
fascicolo: 9, volume: 54, anno: 2000,
pagine: 1828 - 1832
SICI:
0028-3878(20000509)54:9<1828:CIGFII>2.0.ZU;2-#
Fonte:
ISI
Lingua:
ENG
Soggetto:
PALMITOYL-PROTEIN THIOESTERASE; FACTOR-I; RAT-BRAIN; PROGRESSIVE ENCEPHALOPATHY; MRI EVALUATION; OPTIC ATROPHY; PEHO SYNDROME; SURVIVAL; NEURODEGENERATION; APOPTOSIS;
Keywords:
CSF insulin-like growth factor-1; infantile neuronal ceroid lipofuscinosis; apoptosis;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
42
Recensione:
Indirizzi per estratti:
Indirizzo: Riikonen, R Kajavatie 7, Porvoo 06100, Finland Kajavatie 7 Porvoo Finland 06100 ie 7, Porvoo 06100, Finland
Citazione:
R. Riikonen et al., "CSF insulin-like growth factor-1 in infantile neuronal ceroid lipofuscinosis", NEUROLOGY, 54(9), 2000, pp. 1828-1832

Abstract

Background: Infantile neuronal ceroid lipofuscinosis (INCL) is a progressive encephalopathy in which the patients are severely disabled by the age of3 years. It is characterized by cerebral atrophy, selective loss of cortical neurons, and secondary loss of axons and myelin sheaths of the white matter. INCL has been shown to result from a palmitoyl protein thioesterase deficiency. The authors suggested that insulin-like growth hormones and apoptosis might play a role in the pathogenesis of INCL. Methods: The authors measured insulin-like growth factor-1 (IGF-1) and IGF binding protein 3 (IGFBP-3) in the CSF of patients with INCL by radioimmunoassay at an early stagewhen myelin was starting to diminish. Results: The authors found low CSF IGF-1 but normal IGFBP-3 in patients with INCL compared with control subjects. Also, they observed apoptotic cell death in biopsies of INCL patients. Conclusions: Because the IGF system seems to be important for early brain development, myelination, and neuroprotection, the authors suggest that the pathology in INCL may be associated with low CSF IGF-1.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 02/12/20 alle ore 18:29:06