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List of works by Jaana Tyynelä-Vesterinen

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

scientific article published on January 2003

Accumulation of bis(monoacylglycero)phosphate and gangliosides in mouse models of neuronal ceroid lipofuscinosis

scientific article published on 21 May 2008

Accumulation of sphingolipid activator proteins (SAPs) A and D in granular osmiophilic deposits in miniature Schnauzer dogs with ceroid-lipofuscinosis

scientific article

Analysis of phospholipid molecular species in brains from patients with infantile and juvenile neuronal-ceroid lipofuscinosis using liquid chromatography-electrospray ionization mass spectrometry

scientific article published in March 2003

Autosomal dominant adult neuronal ceroid lipofuscinosis: a novel form of NCL with granular osmiophilic deposits without palmitoyl protein thioesterase 1 deficiency.

scientific article

Cathepsin D deficiency induces cytoskeletal changes and affects cell migration pathways in the brain

scientific article published on 12 October 2012

Cathepsin D deficiency underlies congenital human neuronal ceroid-lipofuscinosis

scientific article published on 2 May 2006

Cathepsin D expression level affects alpha-synuclein processing, aggregation, and toxicity in vivo.

scientific article published on 09 February 2009

Cathepsin D-deficient Drosophila recapitulate the key features of neuronal ceroid lipofuscinoses

scientific article

Cell biology and function of neuronal ceroid lipofuscinosis-related proteins ⬇️

scientific article published on 09 February 2013

Characterization of the NF2 protein merlin and the ERM protein ezrin in human, rat, and mouse central nervous system

scientific journal article

Congenital ovine neuronal ceroid lipofuscinosis--a cathepsin D deficiency with increased levels of the inactive enzyme

scientific article published in January 2001

Developmental changes in the expression of neuronal ceroid lipofuscinoses-linked proteins.

scientific article

Different patterns of hydrophobic protein storage in different forms of neuronal ceroid lipofuscinosis (NCL, Batten disease)

scientific article published on 01 February 1997

Distinct neuropathologic phenotypes after disrupting the chloride transport proteins ClC-6 or ClC-7/Ostm1.

scientific article published in December 2010

Drafting the CLN3 protein interactome in SH-SY5Y human neuroblastoma cells: a label-free quantitative proteomics approach.

scientific article

Enhanced activation of bound plasminogen on Staphylococcus aureus by staphylokinase

scientific article

Enhanced expression of manganese-dependent superoxide dismutase in human and sheep CLN6 tissues

scientific article published on December 2003

Exacerbated neuronal ceroid lipofuscinosis phenotype in Cln1/5 double-knockout mice

scientific article published on 12 October 2012

From genes to systems: new global strategies for the characterization of NCL biology

scientific article published on 12 September 2006

Gene expression alterations in the cerebellum and granule neurons of Cstb(-/-) mouse are associated with early synaptic changes and inflammation

scientific article

High-resolution magic angle spinning and 1H magnetic resonance spectroscopy reveal significantly altered neuronal metabolite profiles in CLN1 but not in CLN3.

scientific article published in September 2004

Hippocampal lesions in the neuronal ceroid lipofuscinoses

scientific article published in January 2001

Hippocampal pathology in the human neuronal ceroid-lipofuscinoses: distinct patterns of storage deposition, neurodegeneration and glial activation

scientific article published on 01 October 2004

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

scientific article

Kufs or not Kufs: challenging diagnostics of a rare adult-onset neurodegenerative disease

scientific article published on 01 January 2019

Lysosomal dysfunction promotes cleavage and neurotoxicity of tau in vivo

scientific article

Mice with targeted Slc4a10 gene disruption have small brain ventricles and show reduced neuronal excitability

scientific article

Morphologic and functional correlates of synaptic pathology in the cathepsin D knockout mouse model of congenital neuronal ceroid lipofuscinosis

scientific article

Murine cathepsin D deficiency is associated with dysmyelination/myelin disruption and accumulation of cholesteryl esters in the brain

scientific article published on 20 October 2009

Mutations in DNAJC5, Encoding Cysteine-String Protein Alpha, Cause Autosomal-Dominant Adult-Onset Neuronal Ceroid Lipofuscinosis.

scientific article

Mutations in DNAJC5, encoding cysteine-string protein alpha, cause autosomal-dominant adult-onset neuronal ceroid lipofuscinosis

scientific article

NCL disease mechanisms

scientific article published on 23 May 2013

Northern epilepsy: a novel form of neuronal ceroid-lipofuscinosis

scientific article published in April 2000

Novel mutation and the first prenatal screening of cathepsin D deficiency (CLN10).

scientific article published on 2 September 2008

Palmitoyl protein thioesterase 1 (PPT1) deficiency causes endocytic defects connected to abnormal saposin processing

scientific article

Phosphorylation of a peptide related to subunit c of the F0F1-ATPase/ATP synthase and relationship to permeability transition pore opening in mitochondria

scientific article published in August 2002

Pirkko Santavuori (1933-2004).

scientific article published in June 2004

Potential role of subunit c of F0F1-ATPase and subunit c of storage body in the mitochondrial permeability transition. Effect of the phosphorylation status of subunit c on pore opening

scientific article published on 17 December 2013

Proteomic Profiling in the Brain of CLN1 Disease Model Reveals Affected Functional Modules.

scientific article published on 26 December 2015

Proteomic analysis of the palmitoyl protein thioesterase 1 interactome in SH-SY5Y human neuroblastoma cells.

scientific article

Quantitative analysis of PPT1 interactome in human neuroblastoma cells.

scientific article published on 12 June 2015

Sphingolipid activator proteins (SAPs) are stored together with glycosphingolipids in the infantile neuronal ceroid-lipofuscinosis (INCL)

scientific article published on 01 June 1995

Synaptic Changes in the Thalamocortical System of Cathepsin D-Deficient Mice

scientific article published on 01 January 2008

Synergistic effects of treating the spinal cord and brain in CLN1 disease.

scientific article

The expression of palmitoyl-protein thioesterase is developmentally regulated in neural tissues but not in nonneural tissues

scientific article published in April 1999

Tumor-Associated Trypsinogen-2 (Trypsinogen-2) Activates Procollagenases (MMP-1, -8, -13) and Stromelysin-1 (MMP-3) and Degrades Type I Collagen ⬇️

scientific article published on May 13, 2003

Variant late infantile neuronal ceroid-lipofuscinosis: pathology and biochemistry

scientific article published in April 1997

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