Abstract: The present invention relates to novel bis-hydrazone derivatives of formula (I): wherein Ar1 and Ar2 may be identical or different and are each independently selected from the group consisting of groups of formula (II) and (III): Y1 and Z1 are independently CH or NRc+, provided that al least one of Y1 and Z1 is NRc+ and at least one of Y1 and Z1 is CH, and Ra, Rb, Rc, X2? and L as defined in the claims, or a hydrate or a solvate thereof. Compositions and kits comprising same are also described. Said bis-hydrazone derivatives of formula (I), compositions and kits are useful as drugs, in particular for treating or preventing cancers associated with the Epstein-Barr Virus.

Abstract: The present invention relates to the use of disulfiram or one of its derivatives for use in the treatment of a mitochondrial dysfunction or diseases, advantageously of a genetic mitochondrial disease.

Abstract: The present invention relates to novel bis-hydrazone derivatives of formula (I): wherein Ar1 and Ar2 may be identical or different and are each independently selected from the group consisting of groups of formula (II) and (III): Y1 and Z1 are independently CH or NRc+, provided that al least one of Y1 and Z1 is NRc+ and at least one of Y1 and Z1 is CH, and Ra, Rb, Rc, X2? and L as defined in the claims, or a hydrate or a solvate thereof. Compositions and kits comprising same are also described. Said bis-hydrazone derivatives of formula (I), compositions and kits are useful as drugs, in particular for treating or preventing cancers associated with the Epstein-Barr Virus.

Abstract: The present invention relates to the use of compounds of general formula (I): (I) for the preparation of medicaments that act against mitochondrial pathologies involving a deficiency in ATP production via the oxidative phosphorylation pathway, such as mitochondrial diseases.

Abstract: The invention concerns the use of guanabenz and its derivatives for making drugs for treating cystic fibrosis and diseases related to a protein addressing deficiency in the cells, said derivatives corresponding to formula (I), wherein: R?H or C1 and the phenyl group comprises two substituents, or a pharmaceutically acceptable salt of said derivatives.

Abstract: The invention concerns screening molecules with anti-prion activity. More particularly, it concerns kits for screening molecules with anti-prion activity characterized in that they comprise in combination a [PSI+], phenotype yeast, an antibiogram and an agent for purifying prions at sub-efficient doses, said yeast including the ade1-14 allele of the ADE1 gene and an inactivated ERG6 gene, the screening methods, and a family of molecules with anti-prion activity isolated by the inventive screen. The invention is applicable to anti-prion agents for producing medicines in particular for treating neurodegenerative diseases involving protein aggregates.

Abstract: The invention concerns screening molecules with anti-prion activity. More particularly, it concerns kits for screening molecules with anti-prion activity characterized in that they comprise in combination a [PSI+], phenotype yeast, an antibiogram and an agent for purifying prions at sub-efficient doses, said yeast including the adel-14 allele of the ADE1 gene and an inactivated ERG6 gene, the screening methods, and a family of molecules with anti-prion activity isolated by the inventive screen. The invention is applicable to anti-prion agents for producing medicines in particular for treating neurodegenerative diseases involving protein aggregates.

Abstract: The invention relates to chlorine Guanabenz derivatives for treating prion-based diseases. More specifically, it relates to the use of the molecule of formula (I) wherein R?H or Cl and the phenyl group is at least substituted twice, or a pharmaceutically acceptable salt thereof, for the manufacture of a medicament for treating prion-based diseases.

Abstract: The invention relates to chlorine Guanabenz derivatives for treating Huntington's disease and other polyglutamine expansion associated diseases. More specifically, it relates to the use of the molecule of formula (I) wherein R?H or Cl and the phenyl group is at least substituted twice, or a pharmaceutically acceptable salt thereof, for the manufacture of a medicament for treating polyglutamine expansion associated diseases.

Abstract: A kit and a method for identifying compounds having anti-prion activity are provided. The kit comprises a yeast of phenotype [PSI+]; an antibiogram; and a prion curing agent in a sub-effective dose, wherein the yeast has the adel-14 allele of the ADE1 gene and an inactivated ERG6 gene. Compounds and pharmaceutical compositions having anti-prion activity are also provided, which are useful for treating various neurodegenerative diseases, including polyglutamines expansion associated diseases; Huntington's disease; Kennedy disease; amyotrophic lateral sclerosis; cerebellous autosomic ataxies; dentalorubral-pallidoluysian atrophy; and spino-bulbar amyotrophy.

Abstract: The invention relates to chlorine Guanabenz derivatives for treating Huntington's disease and other polyglutamine expansion associated diseases. More specifically, it relates to the use of the molecule of formula (I) wherein R?H or Cl and the phenyl group is at least substituted twice, or a pharmaceutically acceptable salt thereof, for the manufacture of a medicament for treating polyglutamine expansion associated diseases.

Abstract: The invention concerns the use of guanabenz and its derivatives for making drugs for treating cystic fibrosis and diseases related to a protein addressing deficiency in the cells, said derivatives corresponding to formula (I), wherein: R?H or C1 and the phenyl group comprises two substituents, or a pharmaceutically acceptable salt of said derivatives.

Abstract: A kit and a method for identifying compounds having anti-prion activity are provided. The kit comprises a yeast of phenotype [PSI+]; an antibiogram; and a prion curing agent in a sub-effective dose, wherein the yeast has the adel-14 allele of the ADE1 gene and an inactivated ERG6 gene. Compounds and pharmaceutical compositions having anti-prion activity are also provided, which are useful for treating various neurodegenerative diseases, including polyglutamines expansion associated diseases; Huntington's disease; Kennedy disease; amyotrophic lateral sclerosis; cerebellous autosomic ataxies; dentalorubral-pallidoluysian atrophy; and spino-bulbar amyotrophy.

Abstract: The invention concerns screening molecules with anti-prion activity. More particularly, it concerns kits for screening molecules with anti-prion activity characterized in that they comprise in combination a [PSI+], phenotype yeast, an antibiogram and an agent for purifying prions at sub-efficient doses, said yeast including the adel-14 allele of the ADE1 gene and an inactivated ERG6 gene, the screening methods, and a family of molecules with anti-prion activity isolated by the inventive screen. The invention is applicable to anti-prion agents for producing medicines in particular for treating neurodegenerative diseases involving protein aggregates.