{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"submitter":["Mantuano P"],"funding":["PRIN – MIUR"],"pagination":["1742"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC8615430"],"repository":["biostudies-literature"],"omics_type":["Unknown"],"volume":["11(11)"],"pubmed_abstract":["ROS-activated cSrc tyrosine kinase (TK) promotes the degradation of β-dystroglycan (β-DG), a dystrophin-glycoprotein complex component, which may reinforce damaging signals in Duchenne muscular dystrophy (DMD). Therefore, cSrc-TK represents a promising therapeutic target. In mdx mice, a 4-week subcutaneous treatment with dasatinib (DAS), a pan-Src-TKs inhibitor approved as anti-leukemic agent, increased muscle β-DG, with minimal amelioration of morphofunctional indices. To address possible dose/pharmacokinetic (PK) issues, a new oral DAS/hydroxypropyl(HP)-β-cyclodextrin(CD) complex was developed and chronically administered to mdx mice. The aim was to better assess the role of β-DG in pathology progression, meanwhile confirming DAS mechanism of action over the long-term, along with its eff"],"journal":["Biomolecules"],"pubmed_title":["β-Dystroglycan Restoration and Pathology Progression in the Dystrophic mdx Mouse: Outcome and Implication of a Clinically Oriented Study with a Novel Oral Dasatinib Formulation."],"pmcid":["PMC8615430"],"funding_grant_id":["DPP NL 2015 Grant entitled “Preclinical studies to validate cSrc tyrosine kinase as therapeutic target in Duchenne muscular dystrophy”","projects n. D908ACB4 (S.C.) and 50646C43 (E.C.)","project n. AIM1801289-2 (P.M.)","Prot. 2017FJSM9S_005"],"pubmed_authors":["Boccanegra B","De Bellis M","Cutrignelli A","Sanarica F","Arduino I","Cappellari O","Denora N","Mele A","Conte E","Cirmi S","Lopedota AA","De Luca A","Mantuano P"],"additional_accession":[]},"is_claimable":false,"name":"β-Dystroglycan Restoration and Pathology Progression in the Dystrophic mdx Mouse: Outcome and Implication of a Clinically Oriented Study with a Novel Oral Dasatinib Formulation.","description":"ROS-activated cSrc tyrosine kinase (TK) promotes the degradation of β-dystroglycan (β-DG), a dystrophin-glycoprotein complex component, which may reinforce damaging signals in Duchenne muscular dystrophy (DMD). Therefore, cSrc-TK represents a promising therapeutic target. In mdx mice, a 4-week subcutaneous treatment with dasatinib (DAS), a pan-Src-TKs inhibitor approved as anti-leukemic agent, increased muscle β-DG, with minimal amelioration of morphofunctional indices. To address possible dose/pharmacokinetic (PK) issues, a new oral DAS/hydroxypropyl(HP)-β-cyclodextrin(CD) complex was developed and chronically administered to mdx mice. The aim was to better assess the role of β-DG in pathology progression, meanwhile confirming DAS mechanism of action over the long-term, along with its eff","dates":{"release":"2021-01-01T00:00:00Z","publication":"2021 Nov","modification":"2025-05-29T22:27:37.794Z","creation":"2022-02-11T13:15:06.74Z"},"accession":"S-EPMC8615430","cross_references":{"pubmed":["34827740"],"doi":["10.3390/biom11111742"]}}