Reviewer 2's Review
“This paper provides a detailed characterization of the mouse model of Sanfilippo Syndrome Type B, a lysosomal storage disorder. The researchers found that the MPS IIIB mouse reflects some, but not all, of the disease symptoms observed in humans. In particular, the mutant mouse demonstrates a mild behavioral phenotype, with more overt changes in sensory responses and neuroanatomy. Overall, the paper provides valuable markers for disease progression, which may be useful in the evaluation of possible therapeutic strategies. Issues include clarification of experimental design, appropriate statistical analysis, and need for age-matched experimental groups in some experiments.”
N.B. These are the general comments made by the reviewer when reviewing the originally submitted version of this paper. The manuscript was revised before publication. Specific minor points addressed during revision of the paper are not shown.
