Identification of B6SJL @entity182 ( XXXX ) @entity19 subgroups with different disease progression rates.

@entity1 @entity24869 @entity66 @entity19 @entity73 @entity4053 @entity182 @entity746
UNASSIGNED: Disease progression rates among @entity1 with @entity746 ( @entity746 ) vary greatly. Although the majority of affected individuals survive 3-5 years following diagnosis, some subgroups experience a more rapidly progressing form, surviving less than 1 year, and other subgroups experience slowly progressing forms, surviving nearly 50 years. Genetic heterogeneity and environmental factors pose significant barriers in investigating @entity1 progression rates. Similar to the case for @entity1 , variation in survival within the @entity182 @entity19 has been well documented, but different progression rates have not been investigated. The present study identifies two subgroups of B6SJL @entity182 ( @entity24869 ) @entity19 with different disease progression rates, a @entity4053 ( @entity4053 ) and slow progression group, as evidenced by differences in the rate of motor function decline. In addition, increased disease-associated gene expression within the @entity4053 facial motor nucleus confirmed the presence of a more severe phenotype. We hypothesize that a more @entity66 phenotype could be the result of 1) an earlier onset of axonal disconnection with a consistent degeneration rate or 2) a more severe or accelerated degenerative process. We performed a facial nerve transection axotomy in both @entity182 subgroups prior to disease onset as a method to standardize the axonal disconnection. Instead of leading to comparable gene expression in both subgroups, this standardization did not eliminate the severe phenotype in the @entity4053 facial nucleus, suggesting that the @entity4053 phenotype is the result of a more severe or accelerated degenerative process. We theorize that these @entity182 subgroups are representative of the rapid and @entity73 phenotypes often experienced in @entity746 . J. Comp. Neurol., 2015.    2015 Wiley Periodicals, Inc.
