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01-07-2017 | Correspondence

Regulation of cathepsin D activity by the FTLD protein progranulin

Authors: Xiaolai Zhou, Daniel H. Paushter, Tuancheng Feng, Cara M. Pardon, Christina S. Mendoza, Fenghua Hu

Published in: Acta Neuropathologica | Issue 1/2017

Excerpt

Progranulin (PGRN) protein, encoded by the granulin (GRN) gene, has been recently implicated in several neurodegenerative diseases [2, 5]. While haploinsufficiency of PGRN leads to frontotemporal lobar degeneration (FTLD) [2, 5], the most prevalent form of early onset dementia after Alzheimer’s disease (AD), complete loss of PGRN is known to cause neuronal ceroid lipofuscinosis (NCL) [1, 13], a group of lysosomal storage diseases. PGRN is a secreted glycoprotein of 7.5 granulin repeats [2, 5]. However, within the cell, PGRN is localized to lysosomes through two independent trafficking pathways [8, 17]. Furthermore, GRN is transcriptionally co-regulated with a number of essential lysosomal genes by the transcriptional factor TFEB [3]. While all these studies suggest an essential role of PGRN in regulating lysosomal function, how PGRN does so is still unclear. …

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Title: Regulation of cathepsin D activity by the FTLD protein progranulin
Authors: Xiaolai Zhou
Daniel H. Paushter
Tuancheng Feng
Cara M. Pardon
Christina S. Mendoza
Fenghua Hu
Publication date: 01-07-2017
Publisher: Springer Berlin Heidelberg
Published in: Acta Neuropathologica / Issue 1/2017
Print ISSN: 0001-6322
Electronic ISSN: 1432-0533
DOI: https://doi.org/10.1007/s00401-017-1719-5

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