TBP and spinocerebellar ataxia type 17: Some of these symptoms, namely glucose intolerance and insulin insensitivity, dyslipidemia, muscle wasting, and body weight loss, have been described also in knock-in and transgenic SBMA mice [41,67], as well as in knock-in mice with either pan- or muscle-specific expression of mutant TBP modeling juvenile forms of SCA17 [60].