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1. (WO2019007996) UTILISATION DE COMPLEXES MÉTALLIQUES DE MACROAZAPYRIDINOPHANES DANS LE TRAITEMENT DE MALADIES
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C LAIMS

A metal complex comprising a compound of general formula (I)


Formula (I)

and at least one metal ion, for use in the treatment of diseases related to the accumulation of intracellular deposits, and/or diseases related to defective autophagy and/or defective proteasome activity, wherein said diseases are independently selected from: Huntington disease, P rion disease, Amyloidoses diseases, Focal segmental glomerulosclerosis, diseases related to an Alpha-1 antitrypsin (AAT) deficiency, sphingolipidoses, Niemann-P ick type C disease (NP C), Wolman disease and C holesteryl E ster S torage Diseases (C E S D), glycogen storage diseases, galactosemia, hereditary fructose intolerance, muccopolysaccharidoses, disorders of pyruvate metabolism, phosphoglycerate kinase deficiency, Lafora disease, Adrenoleukodystrophy, Autophagic Vacuolar Myopathies, Danon's disease, C erebral Cavernous Malformation (C C M), G ranular corneal dystrophy type 2 (G C D2), C hronic Kidney Disease (C K D), Bethlem myopathy, Ullrich congenital muscular dystrophy, Myosclerosis, inclusion body myositis, HIV, adenovirus, hepatitis B, Sjogren's syndrome, sepsis or metabolic acidosis.

The metal complex for use, according to claim 1 , wherein said metal ion is a divalent metal ion.

The metal complex for use, according to claim 2, wherein said divalent metal ion is selected from C u(H), Fe(II), Zn (II) or Mn(II).

The metal complex for use, according to claim 3, wherein said divalent metal ion is Mn(II)

The metal complex for use, according to any of claims 1 to 4, wherein the diseases are selected from Lafora disease, Adrenoleukodystrophy or Huntington disease.

C omposition containing an effective amount of a metal complex comprising a compound of general formula (I)


Formula (I)

and at least one metal ion; together with at least one excipient or carrier, for use in the treatment of diseases related to the accumulation of intracellular deposits, and/or diseases related defective autophagy and/or defective proteasome activity, wherein said diseases are independently selected from: Huntington disease, P rion disease, Amyloidoses diseases, Focal segmental glomerulosclerosis, diseases related to an Alpha-1 antitrypsin (AAT) deficiency, sphingolipidoses, Niemann-P ick type C disease (NPC), Wolman disease and C holesteryl E ster Storage Diseases (C E S D), glycogen storage diseases, galactosemia, hereditary fructose intolerance, muccopolysaccharidoses, disorders of pyruvate metabolism, phosphoglycerate kinase deficiency, Lafora disease, Adrenoleukodystrophy, Autophagic Vacuolar Myopathies, Danon's disease, C erebral Cavernous Malformation (C C M), G ranular corneal dystrophy type 2 (G C D2), C hronic Kidney Disease (C K D), Bethlem myopathy, U llrich congenital muscular dystrophy, Myosclerosis, inclusion body myositis, HIV, adenovirus, hepatitis B, Sjogren's syndrome, sepsis or metabolic acidosis.

The composition for use, according to claim 6, wherein said composition further comprises, a second active ingredient.

The composition for use, according to claim 7, wherein said second active ingredient is independently selected from: metformin, a nucleic acid, an antibody, a microR NA, an antioxidant, a polyphenolic compound, a dopaminergic inhibitor, a cholinesterase inhibitor, riluzole or curcumin.

9 The composition for use, according to any of claims 6 to 8, wherein said metal ion is a divalent metal ion.

10. T he composition for use, according to claim 9, wherein said divalent metal ion is selected from C u(H), Fe(II), Ζη(Π) or Mn(II).

1 1. T he composition for use, according to any of the claims 6 to 10, wherein the diseases are selected from Lafora disease, Adrenoleukodytrophy or Huntington disease.