Bernard Brais Selected Research

polyalanine

6/2010	Cross-talk between canonical Wnt signaling and the sirtuin-FoxO longevity pathway to protect against muscular pathology induced by mutant PABPN1 expression in C. elegans.
1/2009	Oculopharyngeal muscular dystrophy: a polyalanine myopathy.
7/2008	Sirtuin inhibition protects from the polyalanine muscular dystrophy protein PABPN1.
5/2008	PABPN1 polyalanine tract deletion and long expansions modify its aggregation pattern and expression.
6/2007	Soluble expanded PABPN1 promotes cell death in oculopharyngeal muscular dystrophy.
9/2006	The dynamism of PABPN1 nuclear inclusions during the cell cycle.
4/2005	PABPN1 overexpression leads to upregulation of genes encoding nuclear proteins that are sequestered in oculopharyngeal muscular dystrophy nuclear inclusions.
4/2005	Transgenic expression of an expanded (GCG)13 repeat PABPN1 leads to weakness and coordination defects in mice.
11/2003	Polymorphism, shared functions and convergent evolution of genes with sequences coding for polyalanine domains.
10/2003	Involvement of the ubiquitin-proteasome pathway and molecular chaperones in oculopharyngeal muscular dystrophy.

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Bernard Brais Research Topics

Disease

13	Oculopharyngeal Muscular Dystrophy 01/2018 - 08/2003
7	Spastic ataxia Charlevoix-Saguenay type 10/2021 - 06/2011
5	Ataxia (Dyssynergia) 01/2021 - 03/2005
4	Neurodegenerative Diseases (Neurodegenerative Disease) 01/2015 - 10/2003
3	Leukoencephalopathies 10/2014 - 09/2011
3	Muscular Dystrophies (Muscular Dystrophy) 02/2010 - 10/2003
2	Inborn Genetic Diseases (Disease, Hereditary) 01/2021 - 11/2003
2	Hypogonadism (Hypergonadotropic Hypogonadism) 01/2021 - 07/2015
2	Anodontia (Hypodontia) 01/2021 - 07/2015
2	Muscular Diseases (Myopathy) 01/2017 - 01/2009
1	Cerebellar Ataxia (Dysmetria) 01/2022
1	Rare Diseases (Rare Disease) 01/2021
1	Infections 01/2020
1	Nemaline Myopathies (Nemaline Myopathy) 01/2020
1	Rhabdomyolysis 01/2020
1	Respiratory Insufficiency (Respiratory Failure) 01/2020
1	Lung Diseases (Lung Disease) 01/2020
1	Glycogen Storage Disease (Glycogenosis) 01/2020
1	Psychotic Disorders (Schizoaffective Disorder) 06/2019
1	Kufor-Rakeb syndrome 06/2019
1	Spastic Ataxia 01/2019
1	Spinal Muscular Atrophy (Progressive Muscular Atrophy) 01/2018
1	Allan-Herndon-Dudley syndrome 09/2015
1	Polyneuropathies (Polyneuropathy) 06/2015
1	Deafness (Deaf Mutism) 11/2014
1	Brown-Vialetto-Van Laere syndrome 11/2014
1	Hereditary Sensory and Autonomic Neuropathies (HSAN) 08/2011
1	Pain (Aches) 10/2010
1	Migraine with Aura (Familial Hemiplegic Migraine) 10/2010
1	Type 2L Limb-Girdle Muscular Dystrophy 02/2010
1	Miyoshi myopathy 02/2010
1	type 2B Limb-girdle muscular dystrophy 02/2010
1	Limb-Girdle Muscular Dystrophies (Limb-Girdle Muscular Dystrophy) 02/2010
1	autosomal dominant Spastic paraplegia 3 06/2007
1	Hereditary Spastic Paraplegia 06/2007
1	Late Onset Disorders 04/2005
1	Apraxias (Dyspraxia) 03/2005
1	autosomal recessive 1 Spinocerebellar ataxia 03/2005
1	Dysarthria (Spastic Dysarthria) 03/2005
1	Alexander Disease (Alexander's Disease) 09/2003

Drug/Important Bio-Agent (IBA)

14	Proteins (Proteins, Gene)FDA Link 01/2019 - 10/2003
10	polyalanineIBA 06/2010 - 10/2003
5	Poly(A)-Binding Protein IIIBA 07/2008 - 04/2005
3	RNA Polymerase IIIIBA 01/2021 - 07/2015
2	EnzymesIBA 01/2021 - 01/2020
2	Peptide Initiation Factors (Initiation Factor)IBA 01/2021 - 08/2014
2	UbiquitinIBA 01/2017 - 10/2003
2	Nuclear Proteins (Protein, Nuclear)IBA 07/2008 - 04/2005
1	PolymersIBA 01/2022
1	Biomarkers (Surrogate Marker)IBA 01/2021
1	Transcription Factor TFIIIBIBA 01/2021
1	GlycogenIBA 01/2020
1	Troponin T (Troponin T1)IBA 01/2020
1	Dantrolene (Dantrium)FDA LinkGeneric 01/2020
1	Glucose (Dextrose)FDA LinkGeneric 01/2020
1	Aripiprazole (Abilify)FDA Link 06/2019
1	Pharmaceutical PreparationsIBA 01/2018
1	Valproic Acid (Depakote)FDA LinkGeneric 01/2018
1	nusinersenIBA 01/2018
1	VimentinIBA 01/2017
1	Phosphotransferases (Kinase)IBA 01/2017
1	Mitogen-Activated Protein KinasesIBA 01/2017
1	Thyroid HormonesIBA 09/2015
1	alpha-N-acetyl-D-glucosaminidase (alpha-N-acetylglucosaminidase)IBA 06/2015
1	Riboflavin (Vitamin B2)FDA LinkGeneric 11/2014
1	Macrophage Colony-Stimulating FactorIBA 10/2014
1	Methionine-tRNA LigaseIBA 01/2012
1	Amino Acyl-tRNA Synthetases (Aminoacyl-tRNA Synthetase)IBA 09/2011
1	Biological ProductsIBA 08/2011
1	Potassium Channels (Potassium Channel)IBA 10/2010
1	Sirtuin 1IBA 06/2010
1	DysferlinIBA 02/2010
1	Chloride Channels (Chloride Channel)IBA 02/2010
1	AnoctaminsIBA 02/2010
1	SirtuinsIBA 07/2008
1	GuanosineIBA 06/2007
1	Poly(A)-Binding Proteins (Poly(A)-Binding Protein)IBA 04/2005
1	alpha-Fetoproteins (alpha-Fetoprotein)IBA 03/2005
1	cisatracurium (Nimbex)FDA Link 02/2005
1	Molecular Chaperones (Chaperone, Molecular)IBA 10/2003
1	Proteasome Endopeptidase Complex (Proteasome)IBA 10/2003
1	Glial Fibrillary Acidic ProteinIBA 09/2003
1	Heterogeneous Nuclear Ribonucleoprotein A1IBA 08/2003
1	Carrier Proteins (Binding Protein)IBA 08/2003

Therapy/Procedure

2	Activities of Daily Living (ADL) 01/2021 - 10/2018
1	Walkers 10/2021
1	Artificial Respiration (Mechanical Ventilation) 01/2020