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In Vertebrates, hypoglycosylation of \u03b1\u2010dystroglycan has been strongly linked to muscular dystrophy phenotypes, some of which also show variable degrees of cognitive impairments, collectively termed dystroglycanopathies. Only a small number of mutations in the dystroglycan gene, leading to the so called primary dystroglycanopathies, has been described so far, as opposed to the ever\u2010growing number of identified secondary or tertiary dystroglycanopathies (caused by genetic abnormalities in glycosyltransferases or in enzymes involved in the synthesis of the carbohydrate building blocks). The few mutations found within the autonomous N\u2010terminal domain of \u03b1\u2010dystroglycan seem to destabilise it to different degrees, without influencing the overall folding and targeting of the dystroglycan complex. On the contrary other mutations, some located at the \u03b1\/\u03b2 interface of the dystroglycan complex, seem to be able to interfere with its maturation, thus compromising its stability and eventually leading to the intracellular engulfment and\/or partial or even total degradation of the dystroglycan uncleaved precursor.<\/jats:p>","DOI":"10.1111\/jcmm.14218","type":"journal-article","created":{"date-parts":[[2019,3,6]],"date-time":"2019-03-06T05:33:25Z","timestamp":1551850405000},"page":"3058-3062","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":39,"title":["A molecular overview of the primary dystroglycanopathies"],"prefix":"10.1111","volume":"23","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4690-8826","authenticated-orcid":false,"given":"Andrea","family":"Brancaccio","sequence":"first","affiliation":[{"name":"School of Biochemistry University of Bristol Bristol UK"},{"name":"Istituto di Chimica del Riconoscimento 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