Defects in sarcolemma repair and skeletal muscle function after injury in a mouse model of Niemann-Pick type A/B disease

Defects in sarcolemma repair and skeletal muscle function after injury in a mouse model of Niemann-Pick type A/B disease

Abstract Background Niemann-Pick disease type A (NPDA), a disease caused by mutations in acid sphingomyelinase (ASM), involves severe neurodegeneration and early death. Intracellular lipid accumulation and plasma membrane alterations are implicated in the pathology. ASM is also linked to the mechani...

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Bibliographic Details
Main Authors: V. Michailowsky, H. Li, B. Mittra, S. R. Iyer, D. A. G. Mazála, M. Corrotte, Y. Wang, E. R. Chin, R. M. Lovering, N. W. Andrews
Format: Article
Language:English
Published: BMC 2019-01-01
Series:Skeletal Muscle
Subjects:
Acid sphingomyelinase
Skeletal muscle
Lysosome
Calcium
Plasma membrane repair
Online Access:http://link.springer.com/article/10.1186/s13395-018-0187-5

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