June 2013
Volume 54, Issue 15
ARVO Annual Meeting Abstract  |   June 2013
Increasing body weight correlates with loss of ERG in mice with Bardet Biedl Syndrome
Author Affiliations & Notes
  • Arlene Drack
    Ophthalmology, Univ of Iowa Hospitals, Iowa City, IA
  • Sajag Bhattarai
    Ophthalmology, Univ of Iowa Hospitals, Iowa City, IA
  • Edwin Stone
    Ophthalmology, Univ of Iowa Hospitals, Iowa City, IA
  • Kai Wang
    Biostatistics, University of Iowa, Iowa City, IA
  • Daniel Gratie
    Ophthalmology, Univ of Iowa Hospitals, Iowa City, IA
  • Elise Heon
    Ophthalmology, Sickkids Toronto, Toronto, ON, Canada
  • Val Sheffield
    Ophthalmology, Univ of Iowa Hospitals, Iowa City, IA
  • Robert Mullins
    Ophthalmology, Univ of Iowa Hospitals, Iowa City, IA
  • Footnotes
    Commercial Relationships Arlene Drack, None; Sajag Bhattarai, None; Edwin Stone, None; Kai Wang, None; Daniel Gratie, None; Elise Heon, None; Val Sheffield, None; Robert Mullins, Alcon Research Ltd (F)
  • Footnotes
    Support None
Investigative Ophthalmology & Visual Science June 2013, Vol.54, 6067. doi:
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      Arlene Drack, Sajag Bhattarai, Edwin Stone, Kai Wang, Daniel Gratie, Elise Heon, Val Sheffield, Robert Mullins; Increasing body weight correlates with loss of ERG in mice with Bardet Biedl Syndrome. Invest. Ophthalmol. Vis. Sci. 2013;54(15):6067.

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      © ARVO (1962-2015); The Authors (2016-present)

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Purpose: Systemic TUDCA slows development of both retinal degeneration and obesity in a mouse model of Bardet Biedl Syndrome (BBS).We analyzed the relationship between body weight and dark-adapted bright flash b-wave (ERG b-wave) in TUDCA and control BBS mice to determine whether body weight might be used as a surrogate endpoint in treatment trials.

Methods: TUDCA was administered subcutaneously twice weekly to Bbs1M390R/M390R mice at 500mg/kg/dose at 6-8 weeks of age (n=5) or 11 weeks of age (n=5).NaHCO3 was administered to littermate controls at the same ages (n=4, n=3).Body weight and ERG b-wave data were collected at P120 and P180.ERG b-wave amplitudes and body weight were compared between groups using a paired linear regression analysis.The correlation coefficient of change in body weight vs. change in ERG b-wave amplitude between 2 time points was calculated for natural history controls (n=12).

Results: Natural history data for Bbs1M390R/M390R mice demonstrated a very significant negative correlation between body weight and ERG b-wave over time (p=0.0004).Animals treated at 6-8 weeks old with TUDCA had a statistically significantly higher b-wave compared to sham treated (p=0.0022).Body weight was significantly lower in TUDCA treated animals than sham (p=0.0004).The correlation coefficient for difference in weight and b-wave between 2 time points in the TUDCA treated group was -0.7, while the correlation coefficient for these parameters in control mice was 0.46.Bbs1M390R/M390R mice treated at the later age of 11 weeks showed higher ERG b-waves and lower body weight than sham treated, but this was not statistically significant.

Conclusions: This study replicates our previously reported findings of less obesity and better ERG b-wave in Bbs1M390R/M390R mice treated with systemic TUDCA.In addition,it demonstrates an inverse relationship between obesity and ERG b-wave amplitude in untreated mice, and a correlation between changes in body weight and b-wave amplitude in treated mice. As human clinical trials using TUDCA to treat BBS in humans are contemplated, body weight may be considered as a study endpoint since it may be easier to measure and may change more quickly than ERG b-wave. References: Drack et al.,TUDCA slows retinal degeneration in two different mouse models of retinitis pigmentosa and prevents obesity in Bardet-Biedl syndrome type 1 mice. IOVS 2012, 5;53(1):100-6.

Keywords: 695 retinal degenerations: cell biology • 696 retinal degenerations: hereditary • 510 electroretinography: non-clinical  

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