Lista de obras - 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

artículo científico publicado en 2003

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

artículo científico publicado en 2008

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

artículo científico publicado en 1997

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

artículo científico publicado en 2003

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

artículo científico publicado en 2003

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

artículo científico

Cathepsin D deficiency underlies congenital human neuronal ceroid-lipofuscinosis

artículo científico publicado en 2006

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

artículo científico publicado en 2009

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

artículo científico publicado en 2005

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

artículo científico publicado el 9 de febrero de 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

artículo científico publicado en 2001

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

artículo científico publicado en 2000

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

artículo científico publicado en 1997

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

artículo científico publicado en 2010

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

artículo científico publicado en 2013

Enhanced activation of bound plasminogen on Staphylococcus aureus by staphylokinase

artículo científico publicado en 2002

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

artículo científico publicado en 2003

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

artículo científico publicado en 2012

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

artículo científico publicado en 2006

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

artículo científico publicado en 2014

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

artículo científico publicado en 2004

Hippocampal lesions in the neuronal ceroid lipofuscinoses

artículo científico publicado en 2001

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

artículo científico publicado en 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

artículo científico publicado en 2007

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

artículo científico publicado en 2019

Lysosomal dysfunction promotes cleavage and neurotoxicity of tau in vivo

artículo científico publicado en 2010

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

artículo científico publicado en 2007

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

artículo científico publicado en 2011

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

artículo científico publicado en 2009

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

artículo científico publicado en 2011

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

artículo científico publicado en 2011

NCL disease mechanisms

artículo científico

Northern epilepsy: a novel form of neuronal ceroid-lipofuscinosis

artículo científico publicado en 2000

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

artículo científico publicado en 2008

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

artículo científico publicado en 2006

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

artículo científico publicado en 2002

Pirkko Santavuori (1933-2004).

artículo científico publicado en 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

artículo científico publicado en 2013

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

artículo científico publicado en 2015

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

artículo científico publicado en 2015

Quantitative analysis of PPT1 interactome in human neuroblastoma cells.

artículo científico publicado en 2015

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

artículo científico publicado en 1995

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

artículo científico publicado en 2008

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

artículo científico publicado en 2017

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

artículo científico publicado en 1999

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

artículo científico publicado el 13 de mayo de 2003

Variant late infantile neuronal ceroid-lipofuscinosis: pathology and biochemistry

artículo científico publicado en 1997

Lista de obras de 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

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

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

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

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

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

Cathepsin D deficiency underlies congenital human neuronal ceroid-lipofuscinosis

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

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

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

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

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

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

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

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

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

Enhanced activation of bound plasminogen on Staphylococcus aureus by staphylokinase

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

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

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

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

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

Hippocampal lesions in the neuronal ceroid lipofuscinoses

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

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

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

Lysosomal dysfunction promotes cleavage and neurotoxicity of tau in vivo

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

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

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

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

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

NCL disease mechanisms

Northern epilepsy: a novel form of neuronal ceroid-lipofuscinosis

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

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

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

Pirkko Santavuori (1933-2004).

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

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

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

Quantitative analysis of PPT1 interactome in human neuroblastoma cells.

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

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

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

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

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

Variant late infantile neuronal ceroid-lipofuscinosis: pathology and biochemistry