title: High-resolution magnetic resonance microscopy and diffusion tensor imaging to assess brain structural abnormalities in the murine mucopolysaccharidosis VII model.
abstract: High-resolution microscopic magnetic resonance imaging (μMRI) and diffusion tensor imaging (DTI) were performed to characterize brain structural abnormalities in a mouse model of mucopolysaccharidosis type VII (MPS VII). Microscopic magnetic resonance imaging demonstrated a decrease in the volume of anterior commissure and corpus callosum and a slight increase in the volume of the hippocampus in MPS VII versus wild-type mice. Diffusion tensor imaging indices were analyzed in gray and white matter. In vivo and ex vivo DTI demonstrated significantly reduced fractional anisotropy in the anterior commissure, corpus callosum, external capsule, and hippocampus in MPS VII versus control brains. Significantly increased mean diffusivity was also found in the anterior commissure and corpus callosum from ex vivo DTI. Significantly reduced linear anisotropy was observed from the hippocampus from in vivo DTI, whereas significantly decreased planar anisotropy and spherical anisotropy were observed in the external capsule from only ex vivo DTI. There were corresponding morphologic differences in the brains of MPS VII mice by hematoxylin and eosin staining. Luxol fast blue staining demonstrated less intense staining of the corpus callosum and external capsule; myelin abnormalities in the corpus callosum were also demonstrated quantitatively in toluidine blue-stained sections and confirmed by electron microscopy. These results demonstrate the potential for μMRI and DTI for quantitative assessment of brain pathology in murine models of brain diseases.
authors: From the Departments of Radiology (MK, IMN, RI, SP, HP), Neurology (JL), and Pediatrics (JHW), Perelman School of Medicine, University of Pennsylvania Philadelphia, Pennsylvania; W.F. Goodman Center for Comparative Medical Genetics, School of Veterinary Medicine, University of Pennsylvania (MKP, JHW); Department of Radiology, New York University School of Medicine, New York, New York (SK); and Research Institute of the Children's Hospital of Philadelphia, Philadelphia, Pennsylvania (MKP, JHW).
authors: Manoj
authors: M
authors: Kumar
authors: Ilya M
authors: IM
authors: Nasrallah
authors: Sungheon
authors: S
authors: Kim
authors: Ranjit
authors: R
authors: Ittyerah
authors: Stephen
authors: S
authors: Pickup
authors: Joel
authors: J
authors: Li
authors: Michael K
authors: MK
authors: Parente
authors: John H
authors: JH
authors: Wolfe
authors: Harish
authors: H
authors: Poptani
mesh_terms: D000818:Animals; D001921:Brain / Q000378:metabolism* / Q000473:pathology* / Q000648:ultrastructure; D056324:Diffusion Tensor Imaging / Q000379:methods*; D004195:Disease Models, Animal; D051379:Mice; D008809:Mice, Inbred C3H; D008854:Microscopy, Electron; D016538:Mucopolysaccharidosis VII / Q000378:metabolism* / Q000473:pathology*