Université Paris-Est, Ecole NationaleVétérinaire d'Alfort, UPR de Neurobiologie, Maisons Alfort, France.
Neuromuscul Disord. 2012 Oct 1;22 Suppl 2:S85-99. doi: 10.1016/j.nmd.2012.05.010.
The Golden Retriever Muscular Dystrophy (GRMD) dog is the closest animal counterpart of Duchenne muscular dystrophy in humans and has, for this reason, increasingly been used in preclinical therapeutic trials for this disease. The aim of this study was to describe the abnormalities in canine dystrophic muscle non-invasively, quantitatively, thoroughly and serially by means of NMR imaging. Thoracic and pelvic limbs of five healthy and five GRMD dogs were imaged in a 3T NMR scanner at 2, 4, 6 and 9months of age. Standard and fat-saturated T(1)-, T(2)- and proton-density-weighted images were acquired. A measurement of T(1) and a two-hour kinetic study of muscle enhancement after gadolinium-chelate injection were also performed. Ten out of the 15 indices evaluated differed between healthy and GRMD dogs. The maximal relative enhancement after gadolinium injection and the proton-density-weighted/T(2)-weighted signal ratio were the most discriminating indices. Inter-muscle heterogeneity was found to vary significantly for most of the indices. The body of data that has been acquired here will help in designing and interpreting preclinical trials using dystrophin-deficient dogs.
金毛寻回猎犬肌肉萎缩症(GRMD)犬是人类中与杜氏肌肉萎缩症最接近的动物模型,因此,它越来越多地被用于该疾病的临床前治疗试验。本研究的目的是通过 NMR 成像非侵入性、定量、全面和连续地描述犬类肌肉萎缩症的异常。在 3T NMR 扫描仪中对 5 只健康和 5 只 GRMD 犬的胸、腿进行成像,分别在 2、4、6 和 9 个月大时进行。采集标准和脂肪饱和 T(1)、T(2)和质子密度加权图像。还进行了 T(1)测量和钆螯合物注射后肌肉增强的两小时动力学研究。在 15 个评估指标中,有 10 个在健康犬和 GRMD 犬之间存在差异。钆注射后的最大相对增强和质子密度加权/T(2)加权信号比是最具区分力的指标。大多数指标的肌肉异质性差异显著。本研究获得的数据将有助于设计和解释使用肌营养不良犬的临床前试验。