List of works - Konstanze Winklhofer

A pathogenic PrP mutation and doppel interfere with polarized sorting of the prion protein.

scientific article

A protein quality control pathway regulated by linear ubiquitination

scientific article published on 18 March 2019

A sensitive filter retention assay for the detection of PrP(Sc) and the screening of anti-prion compounds.

scientific article

Aberrant Folding of Pathogenic Parkin Mutants

scientific article published in Journal of Biological Chemistry

Alpha-synuclein prevents the formation of spherical mitochondria and apoptosis under oxidative stress

scientific article published on 22 February 2017

Alterations in the brain interactome of the intrinsically disordered N-terminal domain of the cellular prion protein (PrPC) in Alzheimer's disease.

scientific article

Amyloid precursor protein elevates fusion of promyelocytic leukemia nuclear bodies in human hippocampal areas with high plaque load

Association of Bcl-2 with misfolded prion protein is linked to the toxic potential of cytosolic PrP.

scientific article published on 17 May 2006

Cationic Lipopolyamines Induce Degradation of PrPSc in Scrapie-Infected Mouse Neuroblastoma Cells

article published in 2000

Cellular prion protein mediates toxic signaling of amyloid beta

scientific article

Cofactor Tpr2 combines two TPR domains and a J domain to regulate the Hsp70/Hsp90 chaperone system

scientific article

Conserved stress-protective activity between prion protein and Shadoo

scientific article published on 21 January 2011

Cytoplasmic protein aggregates interfere with nucleocytoplasmic transport of protein and RNA.

scientific article published on 3 December 2015

Determinants of the in vivo folding of the prion protein. A bipartite function of helix 1 in folding and aggregation

scientific article

Dimerization of the cellular prion protein inhibits propagation of scrapie prions

scientific article published on 10 April 2018

Folding and misfolding of the prion protein in the secretory pathway

scientific article

Geldanamycin restores a defective heat shock response in vivo

scientific article

Green tea extracts interfere with the stress-protective activity of PrP and the formation of PrP.

scientific article

Guidelines for the use and interpretation of assays for monitoring autophagy (3rd edition)

scientific article

Human cytomegalovirus immediate-early gene 2 expression leads to JCV replication in nonpermissive cells via transcriptional activation of JCV T antigen.

scientific article published in September 2000

Impaired complex IV activity in response to loss of LRPPRC function can be compensated by mitochondrial hyperfusion

scientific article

Inactivation of parkin by oxidative stress and C-terminal truncations: a protective role of molecular chaperones

scientific article published on 12 September 2003

Inaktivierung von Parkin bei der Parkinson-Erkrankung

scientific article published on 01 January 2006

Increased levels of mitochondrial import factor Mia40 prevent the aggregation of polyQ proteins in the cytosol

scientific article published on 30 June 2021

Inflammation-induced alteration of astrocyte mitochondrial dynamics requires autophagy for mitochondrial network maintenance

scientific article published in December 2013

Inhibition of complex glycosylation increases the formation of PrPsc

scientific article published in May 2003

Inhibition of mitochondrial fusion by α-synuclein is rescued by PINK1, Parkin and DJ-1.

scientific article published on 14 September 2010

Intracellular re-routing of prion protein prevents propagation of PrP(Sc) and delays onset of prion disease

scientific article

Laquinimod treatment in the R6/2 mouse model

scientific article

Loss of parkin or PINK1 function increases Drp1-dependent mitochondrial fragmentation

scientific article

Loss of the Parkinson's disease-linked gene DJ-1 perturbs mitochondrial dynamics

scientific article

Loss-of-function of human PINK1 results in mitochondrial pathology and can be rescued by parkin

scientific article (publication date: 7 November 2007)

Misfolding of the prion protein at the plasma membrane induces endocytosis, intracellular retention and degradation

scientific article published on June 2004

Mitochondrial dysfunction in Parkinson's disease

scientific article published on 03 September 2009

Mitochondrial dysfunction in Parkinson's disease: molecular mechanisms and pathophysiological consequences

scientific article published on 26 June 2012

Neuroprotective and neurotoxic signaling by the prion protein

scientific article published on January 2011

Neutrophil-generated HOCl leads to non-specific thiol oxidation in phagocytized bacteria.

scientific article

Observing fibrillar assemblies on scrapie-infected cells

PERK activation mitigates tau pathology in vitro and in vivo

scientific article published on February 2017

Parkin and mitochondrial quality control: toward assembling the puzzle

scientific article

Parkin cooperates with GDNF/RET signaling to prevent dopaminergic neuron degeneration

scientific article

Parkin is protective against proteotoxic stress in a transgenic zebrafish model

scientific article

Parkin is transcriptionally regulated by ATF4: evidence for an interconnection between mitochondrial stress and ER stress

scientific article

Parkin mediates neuroprotection through activation of IkappaB kinase/nuclear factor-kappaB signaling

scientific article

Parkin, PINK1 and mitochondrial integrity: emerging concepts of mitochondrial dysfunction in Parkinson's disease

scientific article

Pathogenic mutations inactivate parkin by distinct mechanisms.

scientific article

Pathogenic mutations located in the hydrophobic core of the prion protein interfere with folding and attachment of the glycosylphosphatidylinositol anchor.

scientific article

Pink1-deficiency in mice impairs gait, olfaction and serotonergic innervation of the olfactory bulb

scientific article published on 11 January 2012

Post-translational import of the prion protein into the endoplasmic reticulum interferes with cell viability: a critical role for the putative transmembrane domain

scientific article published on 8 July 2003

Prion protein-related proteins from zebrafish are complex glycosylated and contain a glycosylphosphatidylinositol anchor

scientific article published on 9 January 2006

Protein immobilization on liposomes and lipid-coated nanoparticles by protein trans-splicing

scientific article published on 01 October 2010

Ret rescues mitochondrial morphology and muscle degeneration of Drosophila Pink1 mutants

scientific article published on 28 January 2014

Ret rescues mitochondrial morphology and muscle degeneration of Drosophila Pink1 mutants

scientific article

SecY-mediated quality control prevents the translocation of non-gated porins

scientific article published on 01 October 2020

Semisynthetic murine prion protein equipped with a GPI anchor mimic incorporates into cellular membranes

scientific article published in September 2007

Stress-protective signalling of prion protein is corrupted by scrapie prions

scientific article

Structural features within the nascent chain regulate alternative targeting of secretory proteins to mitochondria

scientific article published on 12 March 2013

Structural instability of the prion protein upon M205S/R mutations revealed by molecular dynamics simulations

scientific article

Synthesis of a GPI anchor module suitable for protein post-translational modification

scientific article published on 01 January 2010

Systematic identification of antiprion drugs by high-throughput screening based on scanning for intensely fluorescent targets

scientific article

TRAP1 rescues PINK1 loss-of-function phenotypes

scientific article published on 21 March 2013

The C-terminal Globular Domain of the Prion Protein Is Necessary and Sufficient for Import into the Endoplasmic Reticulum

scientific article published in Journal of Biological Chemistry

The E3 ligase parkin maintains mitochondrial integrity by increasing linear ubiquitination of NEMO

scientific article

The Sec61/SecY complex is inherently deficient in translocating intrinsically disordered proteins

scientific article published on 30 October 2017

The cellular prion protein mediates neurotoxic signalling of β-sheet-rich conformers independent of prion replication

scientific article published on 25 March 2011

The heat shock response is modulated by and interferes with toxic effects of scrapie prion protein and amyloid β.

scientific article published on 31 October 2012

The key role of solvent in condensation: Mapping water in liquid-liquid phase-separated FUS

scientific article published on 6 April 2021

The mitochondrial chaperone protein TRAP1 mitigates α-Synuclein toxicity

scientific article

The mitochondrial kinase PINK1: functions beyond mitophagy

scientific article published on 2 June 2016

The parkin protein as a therapeutic target in Parkinson's disease

scientific article published on December 2007

The parkin-coregulated gene product PACRG promotes TNF signaling by stabilizing LUBAC

scientific article published on 04 February 2020

The polysaccharide scaffold of PrP 27-30 is a common compound of natural prions and consists of alpha-linked polyglucose

scientific article published in November 2005

The role of chaperones in Parkinson's disease and prion diseases.

scientific article

The two faces of protein misfolding: gain- and loss-of-function in neurodegenerative diseases

scientific article published on January 2008

The α-helical structure of prodomains promotes translocation of intrinsically disordered neuropeptide hormones into the endoplasmic reticulum

scientific article published on 26 March 2013

alpha-Helical domains promote translocation of intrinsically disordered polypeptides into the endoplasmic reticulum

scientific article

List of works by Konstanze Winklhofer

A pathogenic PrP mutation and doppel interfere with polarized sorting of the prion protein.

A protein quality control pathway regulated by linear ubiquitination

A sensitive filter retention assay for the detection of PrP(Sc) and the screening of anti-prion compounds.

Aberrant Folding of Pathogenic Parkin Mutants

Alpha-synuclein prevents the formation of spherical mitochondria and apoptosis under oxidative stress

Alterations in the brain interactome of the intrinsically disordered N-terminal domain of the cellular prion protein (PrPC) in Alzheimer's disease.

Amyloid precursor protein elevates fusion of promyelocytic leukemia nuclear bodies in human hippocampal areas with high plaque load

Association of Bcl-2 with misfolded prion protein is linked to the toxic potential of cytosolic PrP.

Cationic Lipopolyamines Induce Degradation of PrPSc in Scrapie-Infected Mouse Neuroblastoma Cells

Cellular prion protein mediates toxic signaling of amyloid beta

Cofactor Tpr2 combines two TPR domains and a J domain to regulate the Hsp70/Hsp90 chaperone system

Conserved stress-protective activity between prion protein and Shadoo

Cytoplasmic protein aggregates interfere with nucleocytoplasmic transport of protein and RNA.

Determinants of the in vivo folding of the prion protein. A bipartite function of helix 1 in folding and aggregation

Dimerization of the cellular prion protein inhibits propagation of scrapie prions

Folding and misfolding of the prion protein in the secretory pathway

Geldanamycin restores a defective heat shock response in vivo

Green tea extracts interfere with the stress-protective activity of PrP and the formation of PrP.

Guidelines for the use and interpretation of assays for monitoring autophagy (3rd edition)

Human cytomegalovirus immediate-early gene 2 expression leads to JCV replication in nonpermissive cells via transcriptional activation of JCV T antigen.

Impaired complex IV activity in response to loss of LRPPRC function can be compensated by mitochondrial hyperfusion

Inactivation of parkin by oxidative stress and C-terminal truncations: a protective role of molecular chaperones

Inaktivierung von Parkin bei der Parkinson-Erkrankung

Increased levels of mitochondrial import factor Mia40 prevent the aggregation of polyQ proteins in the cytosol

Inflammation-induced alteration of astrocyte mitochondrial dynamics requires autophagy for mitochondrial network maintenance

Inhibition of complex glycosylation increases the formation of PrPsc

Inhibition of mitochondrial fusion by α-synuclein is rescued by PINK1, Parkin and DJ-1.

Intracellular re-routing of prion protein prevents propagation of PrP(Sc) and delays onset of prion disease

Laquinimod treatment in the R6/2 mouse model

Loss of parkin or PINK1 function increases Drp1-dependent mitochondrial fragmentation

Loss of the Parkinson's disease-linked gene DJ-1 perturbs mitochondrial dynamics

Loss-of-function of human PINK1 results in mitochondrial pathology and can be rescued by parkin

Misfolding of the prion protein at the plasma membrane induces endocytosis, intracellular retention and degradation

Mitochondrial dysfunction in Parkinson's disease

Mitochondrial dysfunction in Parkinson's disease: molecular mechanisms and pathophysiological consequences

Neuroprotective and neurotoxic signaling by the prion protein

Neutrophil-generated HOCl leads to non-specific thiol oxidation in phagocytized bacteria.

Observing fibrillar assemblies on scrapie-infected cells

PERK activation mitigates tau pathology in vitro and in vivo

Parkin and mitochondrial quality control: toward assembling the puzzle

Parkin cooperates with GDNF/RET signaling to prevent dopaminergic neuron degeneration

Parkin is protective against proteotoxic stress in a transgenic zebrafish model

Parkin is transcriptionally regulated by ATF4: evidence for an interconnection between mitochondrial stress and ER stress

Parkin mediates neuroprotection through activation of IkappaB kinase/nuclear factor-kappaB signaling

Parkin, PINK1 and mitochondrial integrity: emerging concepts of mitochondrial dysfunction in Parkinson's disease

Pathogenic mutations inactivate parkin by distinct mechanisms.

Pathogenic mutations located in the hydrophobic core of the prion protein interfere with folding and attachment of the glycosylphosphatidylinositol anchor.

Pink1-deficiency in mice impairs gait, olfaction and serotonergic innervation of the olfactory bulb

Post-translational import of the prion protein into the endoplasmic reticulum interferes with cell viability: a critical role for the putative transmembrane domain

Prion protein-related proteins from zebrafish are complex glycosylated and contain a glycosylphosphatidylinositol anchor

Protein immobilization on liposomes and lipid-coated nanoparticles by protein trans-splicing

Ret rescues mitochondrial morphology and muscle degeneration of Drosophila Pink1 mutants

Ret rescues mitochondrial morphology and muscle degeneration of Drosophila Pink1 mutants

SecY-mediated quality control prevents the translocation of non-gated porins

Semisynthetic murine prion protein equipped with a GPI anchor mimic incorporates into cellular membranes

Stress-protective signalling of prion protein is corrupted by scrapie prions

Structural features within the nascent chain regulate alternative targeting of secretory proteins to mitochondria

Structural instability of the prion protein upon M205S/R mutations revealed by molecular dynamics simulations

Synthesis of a GPI anchor module suitable for protein post-translational modification

Systematic identification of antiprion drugs by high-throughput screening based on scanning for intensely fluorescent targets

TRAP1 rescues PINK1 loss-of-function phenotypes

The C-terminal Globular Domain of the Prion Protein Is Necessary and Sufficient for Import into the Endoplasmic Reticulum

The E3 ligase parkin maintains mitochondrial integrity by increasing linear ubiquitination of NEMO

The Sec61/SecY complex is inherently deficient in translocating intrinsically disordered proteins

The cellular prion protein mediates neurotoxic signalling of β-sheet-rich conformers independent of prion replication

The heat shock response is modulated by and interferes with toxic effects of scrapie prion protein and amyloid β.

The key role of solvent in condensation: Mapping water in liquid-liquid phase-separated FUS

The mitochondrial chaperone protein TRAP1 mitigates α-Synuclein toxicity

The mitochondrial kinase PINK1: functions beyond mitophagy

The parkin protein as a therapeutic target in Parkinson's disease

The parkin-coregulated gene product PACRG promotes TNF signaling by stabilizing LUBAC

The polysaccharide scaffold of PrP 27-30 is a common compound of natural prions and consists of alpha-linked polyglucose

The role of chaperones in Parkinson's disease and prion diseases.

The two faces of protein misfolding: gain- and loss-of-function in neurodegenerative diseases

The α-helical structure of prodomains promotes translocation of intrinsically disordered neuropeptide hormones into the endoplasmic reticulum

alpha-Helical domains promote translocation of intrinsically disordered polypeptides into the endoplasmic reticulum