Defective daily temperature regulation in a mouse model of amyotrophic lateral sclerosis.

ABSTRACT: Current understanding of the pathogenesis of the familial form of amyotrophic lateral sclerosis has been aided by the study of transgenic mice that over-express mutated forms of the human CuZn-superoxide dismutase (SOD1) gene. While mutant SOD1 in motor neurons determines disease onset, other non-cell autonomous factors are critical for disease progression, and altered energy metabolism has been implicated as a contributing factor. Since most energy expended by laboratory mice is utilized to defend body temperature (T_b), we analyzed thermoregulation in transgenic mice carrying the G93A mutation of the human SOD1 gene, using implantable temperature data loggers to continuously record T_b for up to 85 days. At room (22 °C) ambient temperature, G93A mice exhibited a dimin

SUBMITTER: Braun MC 

PROVIDER: S-EPMC6263852 | biostudies-literature | 2019 Jan

REPOSITORIES: biostudies-literature