Neurofibromatosis type 1 (NF1) is a multi-system disease caused by mutations in the NF1 gene encoding a Ras-GAP protein, neurofibromin, which negatively regulates Ras signalling. Besides neuroectodermal malformations and tumours, the skeletal system is often affected (e.g. scoliosis and long bone dysplasia), demonstrating the importance of neurofibromin for development and maintenance of the musculoskeletal system. Here we focus on the role of neurofibromin in skeletal muscle development. Nf1 gene inactivation in the early limb bud mesenchyme using Prx1-cre (Nf1Prx1) resulted in muscle dystrophy characterised by fibrosis, reduced number of muscle fibres, and reduced muscle force. To gain insight into the molecular changes of the observed muscle dystrophy and fibrosis and to compare these with other known muscle dystrophies, we performed transcriptional profiling of the entire triceps muscles of threemonth-old wild type (wt) and mutant animals using Affymetrix high-density microrrays.
Neurofibromin (Nf1) is required for skeletal muscle development.
Age, Specimen part
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Differential effects of α-catenin on the invasion and radiochemosensitivity of human colorectal cancer cells.
Cell line
View SamplesThe function of cell-cell contact for radiochemosensitivity is unclear. Here, we investigate the role of the E-cadherin/catenin complex proteins under more physiological three-dimensional (3D) cell culture conditions in a panel of CRC cell lines.
Differential effects of α-catenin on the invasion and radiochemosensitivity of human colorectal cancer cells.
Cell line
View SamplesTo test the effects of narciclasine treatment on major metabolic organs of C57BL/6 mice, we first fed the mice with a high fat diet (HFD) then gavaged them with the narciclasine weekly. After 7 weeks of narciclasine treatment, the four major metabolic organs WAT, BAT, Liver and muscle were harvested and the total RNA was prepared for RNA sequencing analysis. By analyzing the RNA-seq data sets, we found that the primary target of this narciclasine is skeletal muscle. Overall design: Examinaton of expression profile upon narciclasine trearment on different tissues
Narciclasine attenuates diet-induced obesity by promoting oxidative metabolism in skeletal muscle.
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