Sensitivity of ultra-high field MRS to Recovery from Neurodegeneration in a Conditional Mouse Model: A multi-modal investigation with Histology, Behavioral Testing and Quantitative PCR

A conditional transgenic mouse model of spinocerebellar ataxia type 1 (SCA1) was used to determine if ¹ H MRS reflects the extent of neurodegeneration reversal. Transgene expression was suppressed from 12-24 weeks and cerebellar neurochemical profiles of treated and untreated conditional mice and controls were measured at 9.4T using short echo LASER. The data demonstrate that ultra-high field MRS accurately reflects the extent of recovery from neurodegeneration (based on significant correlations with histology and residual transgene expression by qPCR) and is more sensitive than the standard motor behavioral testing (Rotarod) in detecting treatment effects.

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