Proteomic and functional analyses in disease models reveal CLN5 protein involvement in mitochondrial dysfunction.

Neurological disorders Proteomics

Journal

Cell death discovery
ISSN: 2058-7716
Titre abrégé: Cell Death Discov
Pays: United States
ID NLM: 101665035

Informations de publication

Date de publication:
2020
Historique:
received: 03 02 2020
revised: 27 02 2020
accepted: 04 03 2020
entrez: 8 4 2020
pubmed: 8 4 2020
medline: 8 4 2020
Statut: epublish

Résumé

CLN5 disease is a rare form of late-infantile neuronal ceroid lipofuscinosis (NCL) caused by mutations in the

Identifiants

pubmed: 32257390
doi: 10.1038/s41420-020-0250-y
pii: 250
pmc: PMC7105465
doi:

Types de publication

Journal Article

Langues

eng

Pagination

18

Informations de copyright

© The Author(s) 2020.

Déclaration de conflit d'intérêts

Conflict of interestAlessandro Simonati received honoraria for consultancy from Bio-Marin Pharmaceutical, Inc and Neurogene Co; the remaining authors declare that they have no conflict of interest.

Références

J Neurosci Res. 2006 Oct;84(5):1124-38
pubmed: 16941499
CNS Neurosci Ther. 2014 Dec;20(12):1045-55
pubmed: 25230377
BMC Cell Biol. 2009 Nov 26;10:83
pubmed: 19941651
Clin Chim Acta. 2017 Mar;466:46-53
pubmed: 28082023
Sci Rep. 2019 Jan 17;9(1):151
pubmed: 30655561
Front Mol Neurosci. 2017 Aug 22;10:266
pubmed: 28878621
J Proteomics. 2015 Jun 18;123:42-53
pubmed: 25865307
Hum Mol Genet. 2015 Jun 1;24(11):3248-56
pubmed: 25736212
J Neurol. 2015 Dec;262(12):2755-63
pubmed: 26530509
Mol Cell Proteomics. 2019 Nov;18(11):2244-2261
pubmed: 31501224
Mitochondrion. 2012 Mar;12(2):328-35
pubmed: 22310368
Nucleic Acids Res. 2014 Dec 16;42(22):e168
pubmed: 25300484
Essays Biochem. 2017 Dec 12;61(6):733-749
pubmed: 29233882
BMC Neurosci. 2004 Dec 10;5:57
pubmed: 15588329
EMBO J. 2008 Jan 23;27(2):433-46
pubmed: 18200046
Proc Natl Acad Sci U S A. 2019 Jan 2;116(1):277-286
pubmed: 30578322
Acta Neuropathol. 2014;127(6):845-60
pubmed: 24619111
Biochim Biophys Acta. 2015 Oct;1852(10 Pt B):2242-55
pubmed: 25962910
Biochim Biophys Acta. 2013 Nov;1832(11):1807-26
pubmed: 23200925
Sci Rep. 2017 May 9;7(1):1597
pubmed: 28487519
Aging Cell. 2017 Dec;16(6):1276-1287
pubmed: 28884514
Hum Mol Genet. 2014 Apr 15;23(8):2005-22
pubmed: 24271013
Front Pharmacol. 2018 Sep 25;9:1094
pubmed: 30319424
Neurochem Int. 2002 May;40(6):565-71
pubmed: 11850114
Hum Mol Genet. 2001 Jun 15;10(13):1431-9
pubmed: 11440996
Dis Model Mech. 2017 Sep 1;10(9):1089-1100
pubmed: 28733362
Pediatr Res. 2007 Feb;61(2):146-52
pubmed: 17237713
Bioinformatics. 2014 Feb 15;30(4):523-30
pubmed: 24336805
CNS Neurol Disord Drug Targets. 2018;17(9):696-705
pubmed: 30113005
N Engl J Med. 2018 May 17;378(20):1898-1907
pubmed: 29688815
Curr Mol Med. 2014 Oct 13;14(8):1043-1051
pubmed: 25323868
Hum Mol Genet. 2004 Dec 1;13(23):2893-906
pubmed: 15459177
Essays Biochem. 2018 Jul 20;62(3):341-360
pubmed: 30030364
Neurobiol Dis. 2012 Apr;46(1):19-29
pubmed: 22182690
BMC Genomics. 2014 Dec 20;15:1154
pubmed: 25528190
Neurobiol Dis. 2004 Jun;16(1):29-40
pubmed: 15207259
Curr Neurol Neurosci Rep. 2013 Aug;13(8):366
pubmed: 23775425
J Cell Biol. 2016 Dec 5;215(5):649-665
pubmed: 27903607
Nat Methods. 2014 Aug;11(8):783-784
pubmed: 25075903
Clin Immunol. 2018 Dec;197:68-76
pubmed: 30205177
Methods Enzymol. 2014;547:309-54
pubmed: 25416364
Biochem Biophys Res Commun. 2011 Dec 9;416(1-2):159-64
pubmed: 22100646
Front Physiol. 2018 Apr 11;9:365
pubmed: 29695975
Autophagy. 2012 Oct;8(10):1462-76
pubmed: 22889933
Neuromolecular Med. 2016 Mar;18(1):109-33
pubmed: 26707855
Dis Model Mech. 2013 Mar;6(2):342-57
pubmed: 23065637
Biochim Biophys Acta. 2015 Oct;1852(10 Pt B):2237-41
pubmed: 26026925
Dev Med Child Neurol. 2017 Aug;59(8):815-821
pubmed: 28542837
Data Brief. 2015 Jun 12;4:207-16
pubmed: 26217791
Curr Alzheimer Res. 2018;15(14):1283-1296
pubmed: 30289076
Mol Genet Metab. 1999 Apr;66(4):349-55
pubmed: 10191128
Mov Disord. 2012 Sep 15;27(11):1364-9
pubmed: 22927213
BMC Med Genet. 2012 Jun 24;13:50
pubmed: 22727047
Neurobiol Dis. 2009 May;34(2):308-19
pubmed: 19385065
PLoS One. 2013;8(2):e55526
pubmed: 23408996
J Proteome Res. 2013 May 3;12(5):2101-15
pubmed: 23464991
Nat Methods. 2012 Jul;9(7):671-5
pubmed: 22930834
Biochim Biophys Acta. 2015 Oct;1852(10 Pt B):2262-6
pubmed: 25989315
Int J Neurosci. 2001 Aug;108(1-2):109-26
pubmed: 11328706

Auteurs

Stefano Doccini (S)

Molecular Medicine for Neurodegenerative and Neuromuscular Diseases Unit, IRCCS Stella Maris Foundation, Pisa, Italy.

Federica Morani (F)

Molecular Medicine for Neurodegenerative and Neuromuscular Diseases Unit, IRCCS Stella Maris Foundation, Pisa, Italy.

Claudia Nesti (C)

Molecular Medicine for Neurodegenerative and Neuromuscular Diseases Unit, IRCCS Stella Maris Foundation, Pisa, Italy.

Francesco Pezzini (F)

2Neurology (Child Neurology and Neuropathology), Department of Neuroscience, Biomedicine and Movement, University of Verona, Verona, Italy.

Giulio Calza (G)

3Medicum, Biochemistry/Developmental Biology and HiLIFE, Meilahti Clinical Proteomics Core Facility, University of Helsinki, Helsinki, Finland.

Rabah Soliymani (R)

3Medicum, Biochemistry/Developmental Biology and HiLIFE, Meilahti Clinical Proteomics Core Facility, University of Helsinki, Helsinki, Finland.

Giovanni Signore (G)

4NEST, Scuola Normale Superiore, Pisa, Italy.
Fondazione Pisana per la Scienza, Pisa, Italy.

Silvia Rocchiccioli (S)

6Institute of Clinical Physiology (IFC) CNR, Pisa, Italy.

Katja M Kanninen (KM)

7A. I. Virtanen Institute for Molecular Sciences, University of Eastern Finland, Kuopio, Finland.

Mikko T Huuskonen (MT)

7A. I. Virtanen Institute for Molecular Sciences, University of Eastern Finland, Kuopio, Finland.

Marc H Baumann (MH)

3Medicum, Biochemistry/Developmental Biology and HiLIFE, Meilahti Clinical Proteomics Core Facility, University of Helsinki, Helsinki, Finland.

Alessandro Simonati (A)

2Neurology (Child Neurology and Neuropathology), Department of Neuroscience, Biomedicine and Movement, University of Verona, Verona, Italy.

Maciej M Lalowski (MM)

3Medicum, Biochemistry/Developmental Biology and HiLIFE, Meilahti Clinical Proteomics Core Facility, University of Helsinki, Helsinki, Finland.

Filippo M Santorelli (FM)

Molecular Medicine for Neurodegenerative and Neuromuscular Diseases Unit, IRCCS Stella Maris Foundation, Pisa, Italy.

Classifications MeSH