Osteoblast function and bone histomorphometry in a murine model of Rett syndrome

Bone
Mary E BlueJay R Shapiro

Abstract

Rett syndrome (RTT) is an X-linked neurodevelopmental disorder due to mutations affecting the neural transcription factor MeCP2. Approximately 50% of affected females have decreased bone mass. We studied osteoblast function using a murine model of RTT. Female heterozygote (HET) and male Mecp2-null mice were compared to wild type (WT) mice. Micro-CT of tibia from 5 week-old Mecp2-null mice showed significant alterations in trabecular bone including reductions in bone volume fraction (-29%), number (-19%), thickness (-9%) and connectivity density (-32%), and increases in trabecular separation (+28%) compared to WT. We also found significant reductions in cortical bone thickness (-18%) and in polar moment of inertia (-45%). In contrast, cortical and trabecular bone from 8 week-old WT and HET female mice were not significantly different. However, mineral apposition rate, mineralizing surface and bone formation rate/bone surface were each decreased in HET and Mecp2-null mice compared to WT mice. Histomorphometric analysis of femurs showed decreased numbers of osteoblasts but similar numbers of osteoclasts compared to WT, altered osteoblast morphology and decreased tissue synthesis of alkaline phosphatase in Mecp2-null and HET mice. ...Continue Reading

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Citations

Apr 14, 2016·Osteoporosis International : a Journal Established As Result of Cooperation Between the European Foundation for Osteoporosis and the National Osteoporosis Foundation of the USA·M B ZanchettaJ R Zanchetta
Dec 10, 2016·Nature Reviews. Neurology·Helen LeonardJenny Downs
Sep 16, 2020·International Journal of Molecular Sciences·Karin Wuertz-KozakPia M Wippert
Dec 17, 2018·Calcified Tissue International·Arnaud WiedemannFrançois Feillet
Jul 3, 2021·Life·Alessandra PecorelliGiuseppe Valacchi

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