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Titolo:
Palmitoyl protein thioesterase (PPT) localizes into synaptosomes and synaptic vesicles in neurons: implications for infantile neuronal ceroid lipofuscinosis (INCL)
Autore:
Lehtovirta, M; Kyttala, A; Eskelinen, EL; Hess, M; Heinonen, O; Jalanko, A;
Indirizzi:
Natl Publ Hlth Inst, Dept Human Mol Genet, SF-00300 Helsinki, Finland NatlPubl Hlth Inst Helsinki Finland SF-00300 SF-00300 Helsinki, Finland Univ Helsinki, Inst Biotechnol, Electron Microscopy Unit, Helsinki 00710, Finland Univ Helsinki Helsinki Finland 00710 scopy Unit, Helsinki 00710, Finland
Titolo Testata:
HUMAN MOLECULAR GENETICS
fascicolo: 1, volume: 10, anno: 2001,
pagine: 69 - 75
SICI:
0964-6906(20010101)10:1<69:PPT(LI>2.0.ZU;2-#
Fonte:
ISI
Lingua:
ENG
Soggetto:
HIPPOCAMPAL-NEURONS; EXPRESSION; ASPARTYLGLUCOSAMINIDASE; DISEASE; ASSOCIATION; DEFICIENCY; TRANSPORT; MUTATIONS; RECEPTORS; SNAP-25;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
44
Recensione:
Indirizzi per estratti:
Indirizzo: Jalanko, A Natl Publ Hlth Inst, Dept Human Mol Genet, Mannerheimintie 166,SF-00300 Helsinki, Finland Natl Publ Hlth Inst Mannerheimintie 166 Helsinki Finland SF-00300
Citazione:
M. Lehtovirta et al., "Palmitoyl protein thioesterase (PPT) localizes into synaptosomes and synaptic vesicles in neurons: implications for infantile neuronal ceroid lipofuscinosis (INCL)", HUM MOL GEN, 10(1), 2001, pp. 69-75

Abstract

A deficiency of palmitoyl protein thioesterase (PPT) leads to the neurodegenerative disease infantile neuronal ceroid lipofuscinosis (INCL), which ischaracterized by an almost complete loss of cortical neurons. PPT expressed in COS-1 cells is recognized by the mannose-6-phosphate receptor (M6PR) and is routed to lysosome, but a substantial fraction of PPT is secreted. Wehave here determined the neuronal localization of PPT by confocal microscopy, cryoimmunoelectron microscopy and cell fractionation. In mouse primary neurons and brain tissue, PPT is localized in synaptosomes and synaptic vesicles but not in lysosomes, Furthermore, in polarized epithelial Caco-2 cells, PPT is localized exclusively to the basolateral site, in contrast to the classical lysosomal enzyme, aspartylglucosaminidase (AGA), which is localized in the apical site. The current data imply that PPT has a role outsidethe lysosomes in the brain and may be associated with synaptic functioning. This finding operas a new route to study the neuropathological events associated with INCL.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 24/01/20 alle ore 12:20:18