Unravelling a mechanistic link between mitophagy defect, mitochondrial malfunction, and apoptotic neurodegeneration in Mucopolysaccharidosis VII

Cognitive disability and neurodegeneration are prominent symptoms of Mucopolysaccharidosis VII (MPS VII), a lysosomal storage disorder caused by β-glucuronidase enzyme deficiency. Yet, the mechanism of neurodegeneration in MPS VII remains unclear thereby limiting the scope of targeted therapy. We ai...

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Hlavní autoři: Nishan Mandal, Apurba Das, Rupak Datta
Médium: Článek
Jazyk:English
Vydáno: Elsevier 2025-03-01
Edice:Neurobiology of Disease
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On-line přístup:http://www.sciencedirect.com/science/article/pii/S0969996125000415