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Jaana Tyynelä

Research Profile

Disorders

Neuronal Ceroid Lipofuscinosis

Chemicals & Drugs

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Molecular Sequence Data

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Enzo Scifo; Agnieszka Szwajda; Jaana Tyynelä; Kristiina Uusi-Rauva; Marc H Baumann; Michał Dadlez; Janusz Dębski; Anne-Claude Gingras; Anu Jalanko; Tapio Kesti; Maciej Lalowski
Drafting the CLN3 Protein Interactome in SH-SY5Y Human Neuroblastoma Cells: A Label-free Quantitative Proteomics Approach.
Tomas Blom; Jonathan D Cooper; Matti Jauhiainen; Aija Kyttälä; Mia-Lisa Schmiedt; Jarkko Soronen; Jaana Tyynelä; Andrew M Wong; Anu Jalanko
Exacerbated neuronal ceroid lipofuscinosis phenotype in Cln1/5 double-knockout mice.
Sabine Koch; Reeta Korhonen; Maciej Lalowski; Maria Lindfors; Anne Rokka; Enzo Scifo; Pascal Trippner; Garry L Corthals; Ismo Virtanen; Jaana Tyynelä
Cathepsin D deficiency induces cytoskeletal changes and affects cell migration pathways in the brain.

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All Publications

2013: Enzo Scifo; Agnieszka Szwajda; Jaana Tyynelä; Kristiina Uusi-Rauva; Marc H Baumann; Michał Dadlez; Janusz Dębski; Anne-Claude Gingras; Anu Jalanko; Tapio Kesti; Maciej Lalowski
Drafting the CLN3 Protein Interactome in SH-SY5Y Human Neuroblastoma Cells: A Label-free Quantitative Proteomics Approach.
Journal of proteome research 2013;12(5):2101-15.
2013: Tomas Blom; Jonathan D Cooper; Matti Jauhiainen; Aija Kyttälä; Mia-Lisa Schmiedt; Jarkko Soronen; Jaana Tyynelä; Andrew M Wong; Anu Jalanko
Exacerbated neuronal ceroid lipofuscinosis phenotype in Cln1/5 double-knockout mice.
Disease models & mechanisms 2013;6(2):342-57.
2013: Sabine Koch; Reeta Korhonen; Maciej Lalowski; Maria Lindfors; Anne Rokka; Enzo Scifo; Pascal Trippner; Garry L Corthals; Ismo Virtanen; Jaana Tyynelä
Cathepsin D deficiency induces cytoskeletal changes and affects cell migration pathways in the brain.
Neurobiology of disease 2013;50():107-19.
2011: Sabine Koch; Svetlana M Molchanova; Tomi Taira; Jonathan D Cooper; Andrew Edwards; Thomas H Gillingwater; Ann K Wright; Jaana Tyynelä
Morphologic and functional correlates of synaptic pathology in the cathepsin D knockout mouse model of congenital neuronal ceroid lipofuscinosis.
Journal of neuropathology and experimental neurology 2011;70(12):1089-96.
2011: Lenka Nosková; Anna Přistoupilová; Katherine B Sims; John F Staropoli; Viktor Stránecký; Jaana Tyynelä; Christine Van Broeckhoven; Julie van der Zee; Veronika Barešová; Milan Elleder; Hana Hartmannová; Helena Hůlková; Robert Ivanek; Helena Jahnová; Sara E Mole; Peter Nijssen; Stanislav Kmoch
Mutations in DNAJC5, encoding cysteine-string protein alpha, cause autosomal-dominant adult-onset neuronal ceroid lipofuscinosis.
American journal of human genetics 2011;89(2):241-52.
2010: Sarah N R Pressey; Kieran J O'Donnell; Tobias Stauber; Jens C Fuhrmann; Jaana Tyynelä; Thomas J Jentsch; Jonathan D Cooper
Distinct neuropathologic phenotypes after disrupting the chloride transport proteins ClC-6 or ClC-7/Ostm1.
Journal of neuropathology and experimental neurology 2010;69(12):1228-46.
2010: Vikram Khurana; Ilan Elson-Schwab; Tudor A Fulga; Katherine A Sharp; Carin A Loewen; Erin Mulkearns; Jaana Tyynelä; Clemens R Scherzer; Mel B Feany
Lysosomal dysfunction promotes cleavage and neurotoxicity of tau in vivo.
PLoS genetics 2010;6(7):e1001026.
2010: Aino-Liisa Mutka; Aleksi Haapanen; Reijo Kakela; Maria Lindfors; Ann K Wright; Teija Inkinen; Martin Hermansson; Anne Rokka; Garry Corthals; Matti Jauhiainen; Thomas H Gillingwater; Elina Ikonen; Jaana Tyynelä
Murine cathepsin D deficiency is associated with dysmyelination/myelin disruption and accumulation of cholesteryl esters in the brain.
Journal of neurochemistry 2010;112(1):193-203.
2009: Karen Fritchie; Eija Siintola; Diane Armao; Anna-Elina Lehesjoki; Thomas Marino; CM Powell; Michael B Tennison; Jessica K Booker; Sabine Koch; Sanna Partanen; Kinuko Suzuki; Jaana Tyynelä; Leigh B Thorne
Novel mutation and the first prenatal screening of cathepsin D deficiency (CLN10).
Acta neuropathologica 2009;117(2):201-8.
2009: Valerie Cullen; Maria Lindfors; Juliana Ng; Anders Paetau; Erika Swinton; Piotr Kolodziej; Heather Boston; Paul Saftig; John Woulfe; Mel B Feany; Liisa Myllykangas; Michael G Schlossmacher; Jaana Tyynelä
Cathepsin D expression level affects alpha-synuclein processing, aggregation, and toxicity in vivo.
Molecular brain 2009;2():5.
2008: Sabrina Jabs; Arne Quitsch; Reijo Kakela; Bettina Koch; Jaana Tyynelä; Helmut Brade; Markus Glatzel; Steven U Walkley; Paul Saftig; marie T vanier; Thomas Braulke
Accumulation of bis(monoacylglycero)phosphate and gangliosides in mouse models of neuronal ceroid lipofuscinosis.
Journal of neurochemistry 2008;106(3):1415-25.
2008: Laura E Aberg; M Talling; T Härkönen; Tuula Lönnqvist; Mikael Knip; Reija Alen; Heikki Rantala; Jaana Tyynelä
Intermittent prednisolone and autoantibodies to GAD65 in juvenile neuronal ceroid lipofuscinosis.
Neurology 2008;70(14):1218-20.
2008: Stefan Jacobs; Eva Ruusuvuori; Sampsa T Sipilä; Aleksi Haapanen; Helle H Damkier; Ingo Kurth; Moritz Hentschke; Michaela Schweizer; York Rudhard; Linda M Laatikainen; Jaana Tyynelä; Jeppe Praetorius; Juha Voipio; Christian A Hübner
Mice with targeted Slc4a10 gene disruption have small brain ventricles and show reduced neuronal excitability.
Proceedings of the National Academy of Sciences of the United States of America 2008;105(1):311-6.
2008: Sanna Partanen; Aleksi Haapanen; Catherine Kielar; Charles Pontikis; Noreen Alexander; Teija Inkinen; Paul Saftig; Thomas H Gillingwater; Jonathan D Cooper; Jaana Tyynelä
Synaptic changes in the thalamocortical system of cathepsin D-deficient mice: a model of human congenital neuronal ceroid-lipofuscinosis.
Journal of neuropathology and experimental neurology 2008;67(1):16-29.
2007: Aleksi Haapanen; Usama Abo Ramadan; Taina Autti; Raimo Joensuu; Jaana Tyynelä
In vivo MRI reveals the dynamics of pathological changes in the brains of cathepsin D-deficient mice and correlates changes in manganese-enhanced MRI with microglial activation.
Magnetic resonance imaging 2007;25(7):1024-31.
2007: Taina Autti; J Hämäläinen; Laura E Aberg; Leena Lauronen; Jaana Tyynelä; K Van Leemput
Thalami and corona radiata in juvenile NCL (CLN3): a voxel-based morphometric study.
European journal of neurology : the official journal of the European Federation of Neurological Societies 2007;14(4):447-50.
2006: Anu Jalanko; Jaana Tyynelä; Leena Peltonen
From genes to systems: new global strategies for the characterization of NCL biology.
Biochimica et biophysica acta 2006;1762(10):934-44.
2006: Eija Siintola; Sanna Partanen; Petter Strömme; Aleksi Haapanen; Matti Haltia; Jan Maehlen; Anna-Elina Lehesjoki; Jaana Tyynelä
Cathepsin D deficiency underlies congenital human neuronal ceroid-lipofuscinosis.
Brain : a journal of neurology 2006;129(Pt 6):1438-45.
2006: Laura Ahtiainen; Kaisu Luiro; Maria Kauppi; Jaana Tyynelä; Outi Kopra; Anu Jalanko
Palmitoyl protein thioesterase 1 (PPT1) deficiency causes endocytic defects connected to abnormal saposin processing.
Experimental cell research 2006;312(9):1540-53.
2005: Liisa Myllykangas; Jaana Tyynelä; Andrea Page-McCaw; Gerald M Rubin; Matti Haltia; Mel B Feany
Cathepsin D-deficient Drosophila recapitulate the key features of neuronal ceroid lipofuscinoses.
Neurobiology of disease 2005;19(1-2):194-9.
2005: Mikaela Grönholm; Tambet Teesalu; Jaana Tyynelä; Katja Piltti; Tom Böhling; Kirmo Wartiovaara; Antti Vaheri; Olli Carpén
Characterization of the NF2 protein merlin and the ERM protein ezrin in human, rat, and mouse central nervous system.
Molecular and cellular neurosciences 2005;28(4):683-93.
2004: Jaana Tyynelä; Jonathan D Cooper; M Nadeem Khan; Stephen J A Shemilts; Matti Haltia
Hippocampal pathology in the human neuronal ceroid-lipofuscinoses: distinct patterns of storage deposition, neurodegeneration and glial activation.
Brain pathology (Zurich, Switzerland) 2004;14(4):349-57.
2004: Beathe Sitter; Taina Autti; Jaana Tyynelä; Ursula Sonnewald; Tone F Bathen; Johanna Puranen; Pirkko Santavuori; Matti Haltia; Anders Paetau; Tuomo Polvikoski; Ingrid S Gribbestad; Anna-Maija Häkkinen
High-resolution magic angle spinning and 1H magnetic resonance spectroscopy reveal significantly altered neuronal metabolite profiles in CLN1 but not in CLN3.
Journal of neuroscience research 2004;77(5):762-9.
2004: Jaana Tyynelä; Taina Autti; Matti Haltia; Sara E Mole
Pirkko Santavuori (1933-2004).
Journal of child neurology 2004;19(6):465-70.
2003: Claudia Heine; Jaana Tyynelä; Jonathan D Cooper; David N Palmer; Milan Elleder; Alfried Kohlschuetter; Thomas Braulke
Enhanced expression of manganese-dependent superoxide dismutase in human and sheep CLN6 tissues.
The Biochemical journal 2003;376(Pt 2):369-76.
2003: Peter Nijssen; Chantal Ceuterick; Otto P van Diggelen; Milan Elleder; Jean-Jacques Martin; Johannes L J M Teepen; Jaana Tyynelä; Raymund A C Roos
Autosomal dominant adult neuronal ceroid lipofuscinosis: a novel form of NCL with granular osmiophilic deposits without palmitoyl protein thioesterase 1 deficiency.
Brain pathology (Zurich, Switzerland) 2003;13(4):574-81.
2003: Claudine Tardy; Jaana Tyynelä; Andrej Hasilik; Thierry Levade; Nathalie Andrieu-Abadie
Stress-induced apoptosis is impaired in cells with a lysosomal targeting defect but is not affected in cells synthesizing a catalytically inactive cathepsin D.
Cell death and differentiation 2003;10(9):1090-100.
2003: Reijo Kakela; Pentti Somerharju; Jaana Tyynelä
Analysis of phospholipid molecular species in brains from patients with infantile and juvenile neuronal-ceroid lipofuscinosis using liquid chromatography-electrospray ionization mass spectrometry.
Journal of neurochemistry 2003;84(5):1051-65.
2003: Sanna Partanen; Stephan Storch; Hans-Gerhard Löffler; Andrej Hasilik; Jaana Tyynelä; Thomas Braulke
A replacement of the active-site aspartic acid residue 293 in mouse cathepsin D affects its intracellular stability, processing and transport in HEK-293 cells.
The Biochemical journal 2003;369(Pt 1):55-62.
2002: Tamara S Azarashvili; Jaana Tyynelä; Irina V Odinokova; Pavel A Grigorjev; Marc Baumann; Yu V Evtodienko; Nils-Erik L Saris
Phosphorylation of a peptide related to subunit c of the F0F1-ATPase/ATP synthase and relationship to permeability transition pore opening in mitochondria.
Journal of bioenergetics and biomembranes 2002;34(4):279-84.
2002: J Suopanki; Minnamaija Lintunen; Hannele Lahtinen; Matti Haltia; Pertti Panula; M Baumann; Jaana Tyynelä
Status epilepticus induces changes in the expression and localization of endogenous palmitoyl-protein thioesterase 1.
Neurobiology of disease 2002;10(3):247-57.
2002: Tomi Mölkänen; Jaana Tyynelä; Jari Helin; Nisse Kalkkinen; Pentti Kuusela
Enhanced activation of bound plasminogen on Staphylococcus aureus by staphylokinase.
FEBS letters 2002;517(1-3):72-8.
2001: Jaana Tyynelä; Istvan Sohar; David E Sleat; RM Gin; RJ Donnelly; M Baumann; Matti Haltia; Peter Lobel
Congenital ovine neuronal ceroid lipofuscinosis--a cathepsin D deficiency with increased levels of the inactive enzyme.
European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society 2001;5 Suppl A():43-5.
2001: Matti Haltia; Riitta Herva; J Suopanki; M Baumann; Jaana Tyynelä
Hippocampal lesions in the neuronal ceroid lipofuscinoses.
European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society 2001;5 Suppl A():209-11.
2000: J Suopanki; Sanna Partanen; J Ezaki; M Baumann; E Kominami; Jaana Tyynelä
Developmental changes in the expression of neuronal ceroid lipofuscinoses-linked proteins.
Molecular genetics and metabolism 2000;71(1-2):190-4.
2000: Jaana Tyynelä; Istvan Sohar; David E Sleat; RM Gin; RJ Donnelly; M Baumann; Matti Haltia; Peter Lobel
A mutation in the ovine cathepsin D gene causes a congenital lysosomal storage disease with profound neurodegeneration.
The EMBO journal 2000;19(12):2786-92.
2000: Raili Riikonen; Sanna-Leena Vanhanen; Jaana Tyynelä; Pirkko Santavuori; Ursula Turpeinen
CSF insulin-like growth factor-1 in infantile neuronal ceroid lipofuscinosis.
Neurology 2000;54(9):1828-32.
2000: Tamara S Azarashvily; Jaana Tyynelä; M Baumann; Yu V Evtodienko; Nils-Erik L Saris
Ca(2+)-modulated phosphorylation of a low-molecular-mass polypeptide in rat liver mitochondria: evidence that it is identical with subunit c of F(0)F(1)-ATPase.
Biochemical and biophysical research communications 2000;270(3):741-4.
2000: Riitta Herva; Jaana Tyynelä; Aune Hirvasniemi; M Syrjäkallio-Ylitalo; Matti Haltia
Northern epilepsy: a novel form of neuronal ceroid-lipofuscinosis.
Brain pathology (Zurich, Switzerland) 2000;10(2):215-22.
2000: Jaana Tyynelä; J Suopanki
Biochemical aspects of neuronal ceroid lipofuscinoses.
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology 2000;21(3 Suppl):S21-5.
1999: J Suopanki; Jaana Tyynelä; M Baumann; Matti Haltia
Palmitoyl-protein thioesterase, an enzyme implicated in neurodegeneration, is localized in neurons and is developmentally regulated in rat brain.
Neuroscience letters 1999;265(1):53-6.
1999: J Suopanki; Jaana Tyynelä; M Baumann; Matti Haltia
The expression of palmitoyl-protein thioesterase is developmentally regulated in neural tissues but not in nonneural tissues.
Molecular genetics and metabolism 1999;66(4):290-3.
1997: Galina D Mironova; AV Lazareva; Odile Gateau-Roesch; Jaana Tyynelä; Y Pavlov; M Vanier; Nils-Erik L Saris
Oscillating Ca2+-induced channel activity obtained in BLM with a mitochondrial membrane component.
Journal of bioenergetics and biomembranes 1997;29(6):561-9.
1997: Timo Sorsa; Tuula Salo; Erkki Koivunen; Jaana Tyynelä; Yrjö T Konttinen; Ulrich Bergmann; Ari Tuuttila; E Niemi; Olli Teronen; Päivi Heikkilä; H Tschesche; Jari Leinonen; S Osman; Ulf-Hakan Stenman
Activation of type IV procollagenases by human tumor-associated trypsin-2.
The Journal of biological chemistry 1997;272(34):21067-74.
1997: Jaana Tyynelä; J Suopanki; Pirkko Santavuori; M Baumann; Matti Haltia
Variant late infantile neuronal ceroid-lipofuscinosis: pathology and biochemistry.
Journal of neuropathology and experimental neurology 1997;56(4):369-75.
1997: David N Palmer; Jaana Tyynelä; H C van Mil; Valerie J Westlake; Robert D Jolly
Accumulation of sphingolipid activator proteins (SAPs) A and D in granular osmiophilic deposits in miniature Schnauzer dogs with ceroid-lipofuscinosis.
Journal of inherited metabolic disease 1997;20(1):74-84.
1997: Jaana Tyynelä; J Suopanki; M Baumann; Matti Haltia
Sphingolipid activator proteins (SAPs) in neuronal ceroid lipofuscinoses (NCL).
Neuropediatrics 1997;28(1):49-52.
1997: David N Palmer; Robert D Jolly; H C van Mil; Jaana Tyynelä; Valerie J Westlake
Different patterns of hydrophobic protein storage in different forms of neuronal ceroid lipofuscinosis (NCL, Batten disease).
Neuropediatrics 1997;28(1):45-8.
1996: Seppo Meri; T Lehto; C W Sutton; Jaana Tyynelä; M Baumann
Structural composition and functional characterization of soluble CD59: heterogeneity of the oligosaccharide and glycophosphoinositol (GPI) anchor revealed by laser-desorption mass spectrometric analysis.
The Biochemical journal 1996;316 ( Pt 3)():923-35.
1996: P Hildén; K Savolainen; Jaana Tyynelä; M Vuento; P Kuusela
Purification and characterisation of a plasmin-sensitive surface protein of Staphylococcus aureus.
European journal of biochemistry / FEBS 1996;236(3):904-10.
1995: Jaana Tyynelä; M Baumann; M Henseler; K Sandhoff; Matti Haltia
Sphingolipid activator proteins (SAPs) are stored together with glycosphingolipids in the infantile neuronal ceroid-lipofuscinosis (INCL).
American journal of medical genetics 1995;57(2):294-7.
1995: Matti Haltia; Jaana Tyynelä; M Baumann; M Henseler; K Sandhoff
Immunological studies on sphingolipid activator proteins in the neuronal ceroid-lipofuscinoses.
Gerontology 1995;41 Suppl 2():239-48.
1995: Jaana Tyynelä; M Baumann; M Henseler; K Sandhoff; Matti Haltia
Sphingolipid activator proteins in the neuronal ceroid-lipofuscinoses: an immunological study.
Acta neuropathologica 1995;89(5):391-8.
1994: Moshe Finel; A S Majander; Jaana Tyynelä; A M De Jong; Simon P J Albracht; Mårten Wikström
Isolation and characterisation of subcomplexes of the mitochondrial NADH:ubiquinone oxidoreductase (complex I).
European journal of biochemistry / FEBS 1994;226(1):237-42.
1993: Jaana Tyynelä; David N Palmer; M Baumann; Matti Haltia
Storage of saposins A and D in infantile neuronal ceroid-lipofuscinosis.
FEBS letters 1993;330(1):8-12.