[HTML][HTML] The P2X7 purinoceptor in pathogenesis and treatment of dystrophino-and sarcoglycanopathies

DC Gόrecki, RMH Rumney - Current Opinion in Pharmacology, 2023 - Elsevier
Dystrophinopathy and sarcoglycanopathies are incurable diseases caused by mutations in
the genes encoding dystrophin or members of the dystrophin associated protein complex …

P2X7 purinoceptor as a therapeutic target in muscular dystrophies

DC Górecki - Current Opinion in Pharmacology, 2019 - Elsevier
Highlights•Inflammation is a pathological hallmark of muscular dystrophies.•Dystrophin
and/or sarcoglycans loss alter eATP degradation and P2X7 signalling in muscles.•Treatment …

[HTML][HTML] The Role of P2X7 Purinoceptors in the Pathogenesis and Treatment of Muscular Dystrophies

K Zabłocki, DC Górecki - International Journal of Molecular Sciences, 2023 - mdpi.com
Muscular dystrophies are inherited neuromuscular diseases, resulting in progressive
disability and often affecting life expectancy. The most severe, common types are Duchenne …

[HTML][HTML] P2RX7 purinoceptor: a therapeutic target for ameliorating the symptoms of duchenne muscular dystrophy

A Sinadinos, CNJ Young, R Al-Khalidi, A Teti… - PLoS …, 2015 - journals.plos.org
Background Duchenne muscular dystrophy (DMD) is the most common inherited muscle
disease, leading to severe disability and death in young men. Death is caused by the …

Dystrophin: The dead calm of a dogma

DC Górecki - Rare Diseases, 2016 - Taylor & Francis
Duchenne muscular dystrophy (DMD) is the most common inherited muscle disease leading
to severe disability and death of young men. Current interventions are palliative as no …

P2X7 purinoceptor alterations in dystrophic mdx mouse muscles: relationship to pathology and potential target for treatment

CNJ Young, W Brutkowski, CF Lien… - Journal of cellular …, 2012 - Wiley Online Library
Duchenne muscular dystrophy (DMD) is a lethal inherited muscle disorder. Pathological
characteristics of DMD skeletal muscles include, among others, abnormal Ca2+ …

[HTML][HTML] Aberrant adenosine triphosphate release and impairment of P2Y2-Mediated signaling in sarcoglycanopathies

A Benzi, S Baratto, C Astigiano, L Sturla… - Laboratory …, 2023 - Elsevier
Sarcoglycanopathies, limb-girdle muscular dystrophies (LGMD) caused by genetic loss-of-
function of the membrane proteins sarcoglycans (SGs), are characterized by progressive …

[HTML][HTML] P2X7 receptor antagonist reduces fibrosis and inflammation in a mouse model of alpha-sarcoglycan muscular dystrophy

L Raffaghello, E Principi, S Baratto, C Panicucci… - Pharmaceuticals, 2022 - mdpi.com
Limb-girdle muscular dystrophy R3, a rare genetic disorder affecting the limb proximal
muscles, is caused by mutations in the α-sarcoglycan gene (Sgca) and aggravated by an …

[HTML][HTML] P2X7 purinoceptor affects ectopic calcification of dystrophic muscles

RMH Rumney, J Róg, N Chira, AP Kao… - Frontiers in …, 2022 - frontiersin.org
Ectopic calcification (EC) of myofibers is a pathological feature of muscle damage in
Duchenne muscular dystrophy (DMD). Mineralisation of muscle tissue occurs concomitantly …

Enhanced expression of the P2X4 receptor in Duchenne muscular dystrophy correlates with macrophage invasion

D Yeung, R Kharidia, SC Brown, DC Górecki - Neurobiology of disease, 2004 - Elsevier
Following molecular and immunohistochemical analysis of the purinergic P2X4 receptor
subunit in dystrophin-deficient muscle we have identified a distinct subpopulation of P2X4 …