{"id":497,"date":"2014-11-11T12:30:41","date_gmt":"2014-11-11T11:30:41","guid":{"rendered":"http:\/\/www.futur.glycogenoses.org\/?p=497"},"modified":"2016-01-21T17:52:18","modified_gmt":"2016-01-21T16:52:18","slug":"proteine-chaperone-et-enzymotherapie-substitutive","status":"publish","type":"post","link":"https:\/\/www.glycogenoses.org\/st_qlfctn\/proteine-chaperone-et-enzymotherapie-substitutive\/","title":{"rendered":"Prot\u00e9ine chaperone et enzymoth\u00e9rapie substitutive"},"content":{"rendered":"

Une \u00e9tude pilote rapporte l’am\u00e9lioration de l’activit\u00e9 de l’alpha-glucosidase acide quand elle est associ\u00e9e \u00e0 une prot\u00e9ine chaperone.<\/b><\/p>\n

Pour am\u00e9liorer l’efficacit\u00e9 de l’enzymoth\u00e9rapie substitutive dans la maladie de Pompe, les chercheurs utilisent des prot\u00e9ines chaperonnes qui stabilisent l’alpha-glucosidase acide de substitution et augmentent son activit\u00e9.<\/p>\n

Dans un article publi\u00e9 fin juillet 2014, une \u00e9quipe italienne a \u00e9valu\u00e9 les effets de l’association de l’enzymoth\u00e9rapie substitutive avec la prot\u00e9ine chaperone NB-DNJ (pour N-butyldeoxynojirimycin).<\/p>\n

Cette \u00e9tude en ouvert a \u00e9t\u00e9 r\u00e9alis\u00e9e chez 3 personnes atteintes de maladie de Pompe \u00e0 d\u00e9but pr\u00e9coce et 10, de maladie de Pompe \u00e0 d\u00e9but tardif.<\/p>\n

L’activit\u00e9 de l’alpha-glucosidase acide analys\u00e9e sur tache de sang s\u00e9ch\u00e9 a \u00e9t\u00e9 significativement augment\u00e9e \u00e0 12h, 24h et 36h lorsque les patients ont re\u00e7u le double traitement. L’activit\u00e9 est 1,85 fois plus \u00e9lev\u00e9e que lorsque l’enzymoth\u00e9rapie substitutive est prise seule.<\/p>\n

A Chaperone Enhances Blood Alpha-Glucosidase Activity in Pompe Disease Patients Treated with Enzyme Replacement Therapy.<\/b><\/p>\n

Parenti G, Fecarotta S, Marca GL, Rossi B, Ascione S, Donati MA, Morandi LO, Ravaglia S, Pichiecchio A, Ombrone D, Sacchini M, Pasanisi MB, Filippi PD, Danesino C, Della Casa R, Romano A, Mollica C, Rosa M, Agovino T, Nusco E, Porto C, Andria G.<\/p>\n

Mol Ther. 2014 (Juil). Doi\u00a0: 10.1038\/mt.2014.138. [Epub ahead of print]<\/b><\/p>\n

Sources\u00a0: Site AFM-T\u00e9l\u00e9thon, \u00ab\u00a0Prot\u00e9ine chaperone et enzymoth\u00e9rapie substitutive\u00a0\u00bb<\/p>\n

http:\/\/www.afm-telethon.fr\/actualites\/maladie-pompe-proteine-chaperone-enzymotherapie-substitutive-3051<\/a><\/p>\n","protected":false},"excerpt":{"rendered":"

Une \u00e9tude pilote rapporte l’am\u00e9lioration de l’activit\u00e9 de l’alpha-glucosidase acide quand elle est associ\u00e9e \u00e0 une prot\u00e9ine chaperone. Pour am\u00e9liorer l’efficacit\u00e9 de l’enzymoth\u00e9rapie substitutive dans la maladie de Pompe, les chercheurs utilisent des prot\u00e9ines chaperonnes qui stabilisent l’alpha-glucosidase acide de substitution et augmentent son activit\u00e9. Dans un article publi\u00e9 fin juillet 2014, une \u00e9quipe italienneLeer M\u00e1s<\/p>\n","protected":false},"author":7,"featured_media":0,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":[],"categories":[19,14],"tags":[],"_links":{"self":[{"href":"https:\/\/www.glycogenoses.org\/st_qlfctn\/wp-json\/wp\/v2\/posts\/497"}],"collection":[{"href":"https:\/\/www.glycogenoses.org\/st_qlfctn\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/www.glycogenoses.org\/st_qlfctn\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/www.glycogenoses.org\/st_qlfctn\/wp-json\/wp\/v2\/users\/7"}],"replies":[{"embeddable":true,"href":"https:\/\/www.glycogenoses.org\/st_qlfctn\/wp-json\/wp\/v2\/comments?post=497"}],"version-history":[{"count":1,"href":"https:\/\/www.glycogenoses.org\/st_qlfctn\/wp-json\/wp\/v2\/posts\/497\/revisions"}],"predecessor-version":[{"id":498,"href":"https:\/\/www.glycogenoses.org\/st_qlfctn\/wp-json\/wp\/v2\/posts\/497\/revisions\/498"}],"wp:attachment":[{"href":"https:\/\/www.glycogenoses.org\/st_qlfctn\/wp-json\/wp\/v2\/media?parent=497"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/www.glycogenoses.org\/st_qlfctn\/wp-json\/wp\/v2\/categories?post=497"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/www.glycogenoses.org\/st_qlfctn\/wp-json\/wp\/v2\/tags?post=497"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}