title: Dystrophin induced cognitive impairment: mechanisms, models and therapeutic strategies.
abstract: Existence of conserved domains in dystrophin and its associated complexes provide an opportunity to understand the role of dystrophin associated signalling and its association with neuronal metabolism in a variety of model organisms. We critically reviewed the studies till 2013 through established search engines and databases. Thus, we review the role of dystrophin and its isoforms in different animal models at developmental stages in the neuronal metabolism to enhance the therapeutic strategies. Dystrophin interacts with other proteins in such a way that, when affected, it results in co-morbidities including autism and other neuropsychiatric disorders. It is speculated that various signalling molecules may converge to disrupt neuronal metabolism not adequately studied. TGF-β, RhoGAP and CAM mediated signalling molecules are the chief cause of mortalities due to respiratory and cardiac involvement but remain underevaluated targets for cognitive impairment in DMD/BMD. Manipulation of these signalling pathways could be potent intervention in dystrophin induced cognitive impairment while complementary therapeutic approaches may also be helpful in the treatment of cognitive impairment associated with DMD/BMD.
authors.affiliation: Neuroscience Research Lab, Department of Neurology.
authors.forename: Akshay
authors.initials: A
authors.lastname: Anand
authors.affiliation: Neuroscience Research Lab, Department of Neurology.
authors.forename: Rahul
authors.initials: R
authors.lastname: Tyagi
authors.affiliation: Department of Neurosurgery, Postgraduate Institute of Medical Education and Research, Chandigarh, INDIA;
authors.forename: Manju
authors.initials: M
authors.lastname: Mohanty
authors.affiliation: Department of Neurology, Postgraduate Institute of Medical Education and Research, Chandigarh, INDIA;
authors.forename: Manoj
authors.initials: M
authors.lastname: Goyal
authors.affiliation: Diagnostic and Research Laboratory, Department of Anatomy, University of Sri Jayewardenepura, Nugegoda, SRI LANKA.
authors.forename: K Ranil D De
authors.initials: KR
authors.lastname: Silva
authors.affiliation: Diagnostic and Research Laboratory, Department of Anatomy, University of Sri Jayewardenepura, Nugegoda, SRI LANKA.
authors.forename: Nalaka
authors.initials: N
authors.lastname: Wijekoon
keywords: Cognitive impairment; DMD; Dystrophin; Neuronal Metabolism; mdx