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Human Molecular Genetics, 2001, Vol. 10, No. 1 69-75
© 2001 Oxford University Press

Palmitoyl protein thioesterase (PPT) localizes into synaptosomes and synaptic vesicles in neurons: implications for infantile neuronal ceroid lipofuscinosis (INCL)

Maarit Lehtovirta1, Aija Kyttälä1, Eeva-Liisa Eskelinen2, Michael Hess2, Outi Heinonen1 and Anu Jalanko1,+

1National Public Health Institute, Department of Human Molecular Genetics, Mannerheimintie 166, 00300 Helsinki, Finland and 2Electron Microscopy Unit, Institute of Biotechnology, University of Helsinki, PL 56, Viikinkaari 9, 00710 Helsinki, Finland

A deficiency of palmitoyl protein thioesterase (PPT) leads to the neurodegenerative disease infantile neuronal ceroid lipofuscinosis (INCL), which is characterized 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. We have 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 outside the lysosomes in the brain and may be associated with synaptic functioning. This finding opens a new route to study the neuropathological events associated with INCL.

+ To whom correspondence should be addressed. Tel: +358 9 47448392; Fax: +358 9 47448480; Email: anu.jalanko@ktl.fi


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