(19)
(11) EP 2 249 860 B8

(12) CORRECTED EUROPEAN PATENT SPECIFICATION
Note: Bibliography reflects the latest situation

(15) Correction information:
Corrected version no 1 (W1 B1)

(48) Corrigendum issued on:
18.11.2015 Bulletin 2015/47

(45) Mention of the grant of the patent:
14.10.2015 Bulletin 2015/42

(21) Application number: 09707226.8

(22) Date of filing: 05.02.2009
(51) International Patent Classification (IPC): 
A61K 38/13(2006.01)
A61P 21/00(2006.01)
(86) International application number:
PCT/IB2009/000204
(87) International publication number:
WO 2009/098577 (13.08.2009 Gazette 2009/33)

(54)

NON-IMMUNOSUPPRESSIVE CYCLOSPORIN FOR THE TREATMENT OF LIMB-GIRDLE MUSCULAR DYSTROPHY

NICHT IMMUNSUPPRESSIVES CYCLOSPORIN ZUR BEHANDLUNG VON GLIEDERGÜRTEL-MUSKELDYSTROPHIE

CYCLOSPORINE NON IMMUNOSUPPRESSIVE POUR LE TRAITEMENT DE LA DYSTROPHIE MUSCULAIRE DES CEINTURES


(84) Designated Contracting States:
AT BE BG CH CY CZ DE DK EE ES FI FR GB GR HR HU IE IS IT LI LT LU LV MC MK MT NL NO PL PT RO SE SI SK TR
Designated Extension States:
AL BA RS

(30) Priority: 08.02.2008 WO PCT/IB2008/000292

(43) Date of publication of application:
17.11.2010 Bulletin 2010/46

(73) Proprietor: Debiopharm International SA
1002 Lausanne (CH)

(72) Inventor:
  • MOLKENTIN, Jeffery, D.
    Cincinnati OH 45247 (US)

(74) Representative: Grosfillier, Philippe 
ANDRE ROLAND SA Chemin des Charmettes 9 P.O. Box 5107
1002 Lausanne
1002 Lausanne (CH)


(56) References cited: : 
WO-A-2006/072639
   
  • ANGELIN A ET AL: "Mitochondrial dysfunction in the pathogenesis of Ullrich congenital muscular dystrophy and prospective therapy with cyclosporins" PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA 20070116 US, vol. 104, no. 3, 16 January 2007 (2007-01-16), pages 991-996, XP002504969 ISSN: 0027-8424
  • EMERY A E: "The muscular dystrophies" LANCET THE, LANCET LIMITED. LONDON, GB, vol. 359, no. 9307, 23 February 2002 (2002-02-23), pages 687-695, XP004790771 ISSN: 0140-6736
  • MAGNUS J HANSSON ET AL: "The Nonimmunosuppressive Cyclosporin Analogs NIM811 and UNIL025 Display Nanomolar Potencies on Permeability Transition in Brain-Derived Mitochondria" JOURNAL OF BIOENERGETICS AND BIOMEMBRANES, KLUWER ACADEMIC PUBLISHERS-PLENUM PUBLISHERS, NE, vol. 36, no. 4, 1 August 2004 (2004-08-01) , pages 407-413, XP019280564 ISSN: 1573-6881
  • PARSONS STEPHANIE A ET AL: "Genetic disruption of calcineurin improves skeletal muscle pathology and cardiac disease in a mouse model of limb-girdle muscular dystrophy." THE JOURNAL OF BIOLOGICAL CHEMISTRY 30 MAR 2007, vol. 282, no. 13, 30 March 2007 (2007-03-30), pages 10068-10078, XP002504970 ISSN: 0021-9258 cited in the application
  • DANIELE ET AL: "Ins and outs of therapy in limb girdle muscular dystrophies" INTERNATIONAL JOURNAL OF BIOCHEMISTRY AND CELL BIOLOGY, EXETER, GB, vol. 39, no. 9, 1 January 2007 (2007-01-01), pages 1608-1624, XP022168756 ISSN: 1357-2725
  • NAKAYAMA HIROYUKI ET AL: "Ca2+- and mitochondrial-dependent cardiomyocyte necrosis as a primary mediator of heart failure." THE JOURNAL OF CLINICAL INVESTIGATION SEP 2007, vol. 117, no. 9, September 2007 (2007-09), pages 2431-2444, XP002572910 ISSN: 0021-9738
  • MILLAY DOUGLAS P ET AL: "Genetic and pharmacologic inhibition of mitochondrial-dependent necrosis attenuates muscular dystrophy." NATURE MEDICINE APR 2008, vol. 14, no. 4, April 2008 (2008-04), pages 442-447, XP002572911 ISSN: 1546-170X
  • REUTENAUER J ET AL: "Investigation of Debio 025, a cyclophilin inhibitor, in the dystrophic mdx mouse, a model for Duchenne muscular dystrophy." BRITISH JOURNAL OF PHARMACOLOGY OCT 2008, vol. 155, no. 4, October 2008 (2008-10), pages 574-584, XP002572912 ISSN: 0007-1188
   
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