Devastation of bone tissue in the appendicular skeleton parallels the progression of neuromuscular disease.
Document Type
Article
Publication Date
2009
Keywords
Animals, Behavior-Animal, Bone-Density, Bone-Remodeling, Bone-and-Bones, DNA-Binding-Proteins, Disease-Models-Animal, Disease-Progression, Mice, Mutation, Organ-Size, Osteoporosis, Paralysis, Spinal-Muscular-Atrophies-of-Childhood, Statistics-Nonparametric, Transcription-Factors, X-Ray-Microtomography
First Page
215
Last Page
224
JAX Location
see Reprint Collection (a pdf is available)
JAX Source
J Musculoskelet Neuronal Interact 2009 Oct-Dec; 9(4):215-24.
Abstract
A mouse model of spinal muscular atrophy with respiratory distress (SMARD1) was used to study the consequences of neuromuscular degenerative disease on bone quantity and morphology. Histomorphometry and micro-computed tomography were used to assess the cortical and cancellous bone in the tibia, femur and humerus of adult neuromuscular degeneration (nmd) mice (up to 21w) and age-matched wild-type controls (WT). At 21w, the average lengths of the humerus, tibia and femur were 15%, 10%, and 10% shorter in the nmd mice, respectively. The midshaft of the humerus, tibia and femur of nmd mice had 41%, 47% and 34% less cortical bone than the WT. In the humeral, tibial, and femoral metaphyses of the nmd mice, there was 50%, 78%, and 85% less trabecular bone volume, and 58%, 92%, and 94% less trabecular connectivity than the WT. NMD cortical bone had less than half of the 42% active surface measured in the WT, yet the mineral apposition rate of those surfaces were similar between strains (nmd: 1.80 microm x day(-1); WT: 2.05 microm x day(-1)). Osteoclast number and activity levels did not differ across strains. These data emphasize that neuromuscular degeneration as a result of immunoglobulin S-mu binding protein-2 (Ighmbp2) mutation will compromise several critical parameters of bone quantity and architecture, the most severe occurring in the trabecular compartment.
Recommended Citation
Lee BJ,
Cox GA,
Maddatu TP,
Judex S,
Rubin CT.
Devastation of bone tissue in the appendicular skeleton parallels the progression of neuromuscular disease. J Musculoskelet Neuronal Interact 2009 Oct-Dec; 9(4):215-24.