Title

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

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.

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