List of works - Yvon Trottier

A novel function of Huntingtin in the cilium and retinal ciliopathy in Huntington's disease mice

scientific article

A preliminary study on early onset schizophrenia and bipolar disorder: large polyglutamine expansions are not involved

scientific article published on 01 September 1997

An expanded CAG repeat sequence in spinocerebellar ataxia type 7

scientific article published on 01 October 1996

An expanding story

scientific article published on 01 November 1993

An isoform of ataxin-3 accumulates in the nucleus of neuronal cells in affected brain regions of SCA3 patients.

scientific article published on October 1998

Antibody-based detection of CAG repeat expansion containing genes

article

Biomedicine. Huntingtin--profit and loss.

scientific article

Cellular localization of the Huntington's disease protein and discrimination of the normal and mutated form

scientific journal article

Clinical and genetic analysis of three German kindreds with autosomal dominant cerebellar ataxia type I linked to the SCA2 locus

scientific article published in April 1997

Cloning of the gene for spinocerebellar ataxia 2 reveals a locus with high sensitivity to expanded CAG/glutamine repeats

scientific article

Differential distribution of the normal and mutated forms of huntingtin in the human brain

scientific article published in November 1997

Disease progression despite early loss of polyglutamine protein expression in SCA7 mouse model.

scientific article published in February 2004

Distribution of frataxin in eye retina of normal mice and of transgenic R7E mice with retinal degeneration

scientific article published on July 2010

Domain architecture of the polyglutamine protein ataxin-3: a globular domain followed by a flexible tail

scientific article published in August 2003

Early and reversible neuropathology induced by tetracycline-regulated lentiviral overexpression of mutant huntingtin in rat striatum

scientific article published on 2 September 2003

Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice

scientific article (publication date: November 1996)

Frataxin is reduced in Friedreich ataxia patients and is associated with mitochondrial membranes

scientific article

Gain and loss of function mutations of ataxin-7 cause cilia pathology in mouse and zebrafish models.

scientific article published on 13 July 2015

Glutamine-expanded ataxin-7 alters TFTC/STAGA recruitment and chromatin structure leading to photoreceptor dysfunction.

scientific article

Heterogeneous intracellular localization and expression of ataxin-3.

scientific article published in November 1998

Hsp70 and Hsp40 chaperones do not modulate retinal phenotype in SCA7 mice.

scientific article

Huntingtin affinity for partners is not changed by polyglutamine length: aggregation itself triggers aberrant interactions.

scientific article published on 25 April 2011

Intranuclear aggregation of nonexpanded ataxin-3 in marinesco bodies of the nonhuman primate substantia nigra.

scientific article published in July 2002

Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3.

scientific article published in August 1997

Kinds of mutations induced by aflatoxin B1 in a shuttle vector replicating in human cells transiently expressing cytochrome P450IA2 cDNA

article

Linear and extended: a common polyglutamine conformation recognized by the three antibodies MW1, 1C2 and 3B5H10

scientific article

Linkage disequilibrium between the spinocerebellar ataxia 3/Machado-Joseph disease mutation and two intragenic polymorphisms, one of which, X359Y, affects the stop codon

scientific article published in June 1997

Loss of zebrafish Ataxin-7, a SAGA subunit responsible for SCA7 retinopathy, causes ocular coloboma and malformation of photoreceptors

scientific article published on 01 March 2019

Male with typical fragile X phenotype is deleted for part of the FMR1 gene and for about 100 kb of upstream region.

scientific article published in July 1994

Mapping of the epitope of monoclonal antibody 2B4 to the proline-rich region of human Huntingtin, a region critical for aggregation and toxicity.

scientific article

Molecular Mechanisms and Therapeutic Strategies in Spinocerebellar Ataxia Type 7.

scientific article published in January 2018

Molecular Targets and Therapeutic Strategies in Spinocerebellar Ataxia Type 7

scientific article published on 01 October 2019

Myelinosomes act as natural secretory organelles in Sertoli cells to prevent accumulation of aggregate-prone mutant Huntingtin and CFTR.

scientific article published on 4 August 2016

Neurodegenerative polyglutamine expansion diseases: physiopathology and therapeutic strategies

scientific article published on 29 March 2010

Neuronal distribution of intranuclear inclusions in Huntington's disease with adult onset.

scientific article published in June 1998

PML nuclear bodies and neuronal intranuclear inclusion in polyglutamine diseases

scientific article

Pathogenic and Non-pathogenic Polyglutamine Tracts Have Similar Structural Properties: Towards a Length-dependent Toxicity Gradient

article

Pathological mechanisms in polyglutamine expansion diseases.

scientific article published in January 2001

Polyglutamine expansion as a pathological epitope in Huntington's disease and four dominant cerebellar ataxias

scientific article published on November 1995

Polyglutamine expansion causes neurodegeneration by altering the neuronal differentiation program

scientific article published on 24 January 2006

Polyglutamine expansions and neurodegenerative diseases

scientific article published on January 1, 1996

Polyglutamine toxicity induces rod photoreceptor division, morphological transformation or death in spinocerebellar ataxia 7 mouse retina.

scientific article published on 18 June 2010

Preventing polyglutamine-induced activation of c-Jun delays neuronal dysfunction in a mouse model of SCA7 retinopathy

scientific article published on 26 December 2006

Properties of polyglutamine expansion in vitro and in a cellular model for Huntington's disease.

scientific article

Proteases acting on mutant huntingtin generate cleaved products that differentially build up cytoplasmic and nuclear inclusions

scientific article published on August 2002

Proteolysis of mutant huntingtin produces an exon 1 fragment that accumulates as an aggregated protein in neuronal nuclei in Huntington disease

scientific article

Rat liver cytochrome P450IA2 synthesized by transfected COS-1 cells efficiently activates food-derived promutagens.

scientific article

SCA7 Mouse Cerebellar Pathology Reveals Preferential Downregulation of Key Purkinje Cell-Identity Genes and Shared Disease Signature with SCA1 and SCA2

scientific article published on 22 April 2021

SCA8 CAG/CTG expansions, a tale of two TOXICities: a unique or common case?

scientific article

Screening for proteins with polyglutamine expansions in autosomal dominant cerebellar ataxias

scientific article published on 01 December 1996

Solution structure of polyglutamine tracts in GST-polyglutamine fusion proteins

scientific article published in February 2002

Spinocerebellar ataxia type 7 (SCA7): a neurodegenerative disorder with neuronal intranuclear inclusions

scientific article published in May 1998

Stoichiometry of base excision repair proteins correlates with increased somatic CAG instability in striatum over cerebellum in Huntington's disease transgenic mice

scientific article

Structure of a single-chain Fv bound to the 17 N-terminal residues of huntingtin provides insights into pathogenic amyloid formation and suppression.

scientific article published on 8 April 2015

SynAggreg: A Multifunctional High-Throughput Technology for Precision Study of Amyloid Aggregation and Systematic Discovery of Synergistic Inhibitor Compounds

scientific article published on 25 September 2018

Targeting expression of expanded polyglutamine proteins to the endoplasmic reticulum or mitochondria prevents their aggregation.

scholarly article

The detection of promutagen activation by extracts of cells expressing cytochrome P450IA2 cDNA: preincubation dramatically increases revertant yield in the Ames test.

scientific article published in January 1992

The gene for the TATA binding protein (TBP) that contains a highly polymorphic protein coding CAG repeat maps to 6q27.

scientific article published on June 1994

The nucleotide sequence, DNA damage location, and protein stoichiometry influence the base excision repair outcome at CAG/CTG repeats

scientific article

Transcription Elongation and Tissue-Specific Somatic CAG Instability ⬇️

scientific article published on November 29, 2012

Transcriptional activation of REST by Sp1 in Huntington's disease models

scientific article

Two populations of neuronal intranuclear inclusions in SCA7 differ in size and promyelocytic leukaemia protein content

scientific article

List of works by Yvon Trottier

A novel function of Huntingtin in the cilium and retinal ciliopathy in Huntington's disease mice

A preliminary study on early onset schizophrenia and bipolar disorder: large polyglutamine expansions are not involved

An expanded CAG repeat sequence in spinocerebellar ataxia type 7

An expanding story

An isoform of ataxin-3 accumulates in the nucleus of neuronal cells in affected brain regions of SCA3 patients.

Antibody-based detection of CAG repeat expansion containing genes

Biomedicine. Huntingtin--profit and loss.

Cellular localization of the Huntington's disease protein and discrimination of the normal and mutated form

Clinical and genetic analysis of three German kindreds with autosomal dominant cerebellar ataxia type I linked to the SCA2 locus

Cloning of the gene for spinocerebellar ataxia 2 reveals a locus with high sensitivity to expanded CAG/glutamine repeats

Differential distribution of the normal and mutated forms of huntingtin in the human brain

Disease progression despite early loss of polyglutamine protein expression in SCA7 mouse model.

Distribution of frataxin in eye retina of normal mice and of transgenic R7E mice with retinal degeneration

Domain architecture of the polyglutamine protein ataxin-3: a globular domain followed by a flexible tail

Early and reversible neuropathology induced by tetracycline-regulated lentiviral overexpression of mutant huntingtin in rat striatum

Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice

Frataxin is reduced in Friedreich ataxia patients and is associated with mitochondrial membranes

Gain and loss of function mutations of ataxin-7 cause cilia pathology in mouse and zebrafish models.

Glutamine-expanded ataxin-7 alters TFTC/STAGA recruitment and chromatin structure leading to photoreceptor dysfunction.

Heterogeneous intracellular localization and expression of ataxin-3.

Hsp70 and Hsp40 chaperones do not modulate retinal phenotype in SCA7 mice.

Huntingtin affinity for partners is not changed by polyglutamine length: aggregation itself triggers aberrant interactions.

Intranuclear aggregation of nonexpanded ataxin-3 in marinesco bodies of the nonhuman primate substantia nigra.

Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3.

Kinds of mutations induced by aflatoxin B1 in a shuttle vector replicating in human cells transiently expressing cytochrome P450IA2 cDNA

Linear and extended: a common polyglutamine conformation recognized by the three antibodies MW1, 1C2 and 3B5H10

Linkage disequilibrium between the spinocerebellar ataxia 3/Machado-Joseph disease mutation and two intragenic polymorphisms, one of which, X359Y, affects the stop codon

Loss of zebrafish Ataxin-7, a SAGA subunit responsible for SCA7 retinopathy, causes ocular coloboma and malformation of photoreceptors

Male with typical fragile X phenotype is deleted for part of the FMR1 gene and for about 100 kb of upstream region.

Mapping of the epitope of monoclonal antibody 2B4 to the proline-rich region of human Huntingtin, a region critical for aggregation and toxicity.

Molecular Mechanisms and Therapeutic Strategies in Spinocerebellar Ataxia Type 7.

Molecular Targets and Therapeutic Strategies in Spinocerebellar Ataxia Type 7

Myelinosomes act as natural secretory organelles in Sertoli cells to prevent accumulation of aggregate-prone mutant Huntingtin and CFTR.

Neurodegenerative polyglutamine expansion diseases: physiopathology and therapeutic strategies

Neuronal distribution of intranuclear inclusions in Huntington's disease with adult onset.

PML nuclear bodies and neuronal intranuclear inclusion in polyglutamine diseases

Pathogenic and Non-pathogenic Polyglutamine Tracts Have Similar Structural Properties: Towards a Length-dependent Toxicity Gradient

Pathological mechanisms in polyglutamine expansion diseases.

Polyglutamine expansion as a pathological epitope in Huntington's disease and four dominant cerebellar ataxias

Polyglutamine expansion causes neurodegeneration by altering the neuronal differentiation program

Polyglutamine expansions and neurodegenerative diseases

Polyglutamine toxicity induces rod photoreceptor division, morphological transformation or death in spinocerebellar ataxia 7 mouse retina.

Preventing polyglutamine-induced activation of c-Jun delays neuronal dysfunction in a mouse model of SCA7 retinopathy

Properties of polyglutamine expansion in vitro and in a cellular model for Huntington's disease.

Proteases acting on mutant huntingtin generate cleaved products that differentially build up cytoplasmic and nuclear inclusions

Proteolysis of mutant huntingtin produces an exon 1 fragment that accumulates as an aggregated protein in neuronal nuclei in Huntington disease

Rat liver cytochrome P450IA2 synthesized by transfected COS-1 cells efficiently activates food-derived promutagens.

SCA7 Mouse Cerebellar Pathology Reveals Preferential Downregulation of Key Purkinje Cell-Identity Genes and Shared Disease Signature with SCA1 and SCA2

SCA8 CAG/CTG expansions, a tale of two TOXICities: a unique or common case?

Screening for proteins with polyglutamine expansions in autosomal dominant cerebellar ataxias

Solution structure of polyglutamine tracts in GST-polyglutamine fusion proteins

Spinocerebellar ataxia type 7 (SCA7): a neurodegenerative disorder with neuronal intranuclear inclusions

Stoichiometry of base excision repair proteins correlates with increased somatic CAG instability in striatum over cerebellum in Huntington's disease transgenic mice

Structure of a single-chain Fv bound to the 17 N-terminal residues of huntingtin provides insights into pathogenic amyloid formation and suppression.

SynAggreg: A Multifunctional High-Throughput Technology for Precision Study of Amyloid Aggregation and Systematic Discovery of Synergistic Inhibitor Compounds

Targeting expression of expanded polyglutamine proteins to the endoplasmic reticulum or mitochondria prevents their aggregation.

The detection of promutagen activation by extracts of cells expressing cytochrome P450IA2 cDNA: preincubation dramatically increases revertant yield in the Ames test.

The gene for the TATA binding protein (TBP) that contains a highly polymorphic protein coding CAG repeat maps to 6q27.

The nucleotide sequence, DNA damage location, and protein stoichiometry influence the base excision repair outcome at CAG/CTG repeats

Transcription Elongation and Tissue-Specific Somatic CAG Instability ⬇️

Transcriptional activation of REST by Sp1 in Huntington's disease models

Two populations of neuronal intranuclear inclusions in SCA7 differ in size and promyelocytic leukaemia protein content