Excitation-Contraction Coupling Disruption in a Mouse Model of Niemann-Pick Disease

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dc.contributor.advisorChin, Eva Ren_US
dc.contributor.authorLi, Harry Zichenen_US
dc.contributor.departmentKinesiologyen_US
dc.contributor.publisherDigital Repository at the University of Marylanden_US
dc.contributor.publisherUniversity of Maryland (College Park, Md.)en_US
dc.date.accessioned2017-09-13T05:33:34Z
dc.date.available2017-09-13T05:33:34Z
dc.date.issued2017en_US
dc.description.abstractNiemann-Pick disease (NPD) is a lysosomal storage disorder that results from deficient acid sphingomyelinase (ASM) activity. It was recently proposed that ASM and extracellular Ca2+ are required for membrane repair. Since plasma membrane integrity is an important component of excitation-contraction coupling (E-C) and skeletal muscle force production, we hypothesized that there would be E-C coupling defects in NPD related to intracellular calcium (Ca2+) dynamics. Our results demonstrate that ASM deficient (ASM-/-) fibers have a reduced ability to withstand repetitive contractions in comparison to wild-type (WT) fibers, and fibers from ASM-/- mice exhibited lower peak tetanic Ca2+ compared to WT. Lastly, no differences in peak tetanic Ca2+ were found between ASM-/- fibers and WT fibers deprived of Ca2+. Together, these results suggest that both ASM and extracellular Ca2+ are required for optimal E-C coupling in skeletal muscle and for the ability to respond to repetitive contractions that occurs with sustained activity.en_US
dc.identifierhttps://doi.org/10.13016/M2XK84Q8V
dc.identifier.urihttp://hdl.handle.net/1903/19781
dc.language.isoenen_US
dc.subject.pqcontrolledKinesiologyen_US
dc.subject.pqcontrolledMolecular biologyen_US
dc.subject.pqcontrolledCellular biologyen_US
dc.subject.pquncontrolledacid sphingomyelinaseen_US
dc.subject.pquncontrolledexcitation-contraction couplingen_US
dc.subject.pquncontrolledextracellular calciumen_US
dc.subject.pquncontrolledfatigueen_US
dc.subject.pquncontrolledmembrane repairen_US
dc.subject.pquncontrolledNiemann-Pick diseaseen_US
dc.titleExcitation-Contraction Coupling Disruption in a Mouse Model of Niemann-Pick Diseaseen_US
dc.typeThesisen_US

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