title: TUDCA slows retinal degeneration in two different mouse models of retinitis pigmentosa and prevents obesity in Bardet-Biedl syndrome type 1 mice.
abstract: OBJECTIVE
  To evaluate and compare the protective effect of tauroursodeoxycholic acid (TUDCA) on photoreceptor degeneration in different models of retinal degeneration (RD) in mice.
  
  
  METHODS
  Bbs(M390R/M390R) mice were injected subcutaneously twice a week, from P40 to P120, and rd10 mice were injected every 3 days from P6 to P38 with TUDCA or vehicle (0.15 M NaHCO(3)). Rd1 and rd16 mice were injected daily from P6 to P30 with TUDCA or vehicle. Retinal structure and function were determined at multiple time points by electroretinography (ERG), optical coherence tomography (OCT), and histology.
  
  
  RESULTS
  The amplitude of ERG b-waves was significantly higher in TUDCA-treated Bbs1 and rd10 animals than in controls. Retinal thickness on OCT was slightly greater in treated Bbs1 animals than in the controls. Histologically, outer segments were preserved, and the outer nuclear layer was significantly thicker in the treated Bbs1 and rd10 mice than in the controls. Bbs1(M390R/M390R) mice developed less obesity than the control Bbs1(M390R/M390R) while receiving TUDCA. The Rd1 and rd16 mice showed no improvement with TUDCA treatment, and the rd1 mice did not have normal weight gain during treatment.
  
  
  CONCLUSIONS
  TUDCA treatment preserved ERG b-waves and the outer nuclear layer in Bbs1(M390R/M390R) mice, and prevented obesity assessed at P120. TUDCA treatment preserved ERG b-waves and the outer nuclear layer in the rd10 mice to P30. TUDCA is a prime candidate for treatment of humans with retinal degeneration, especially those with Bardet-Biedl syndrome, whom it may help not only with the vision loss, but with the debilitating obesity as well.
authors: Department of Ophthalmology and Visual Sciences, University of Iowa, Iowa City, Iowa 52242, USA.
authors: Arlene V
authors: AV
authors: Drack
authors: Alina V
authors: AV
authors: Dumitrescu
authors: Sajag
authors: S
authors: Bhattarai
authors: Daniel
authors: D
authors: Gratie
authors: Edwin M
authors: EM
authors: Stone
authors: Robert
authors: R
authors: Mullins
authors: Val C
authors: VC
authors: Sheffield
mesh_terms: D000818:Animals; D020788:Bardet-Biedl Syndrome / Q000188:drug therapy* / Q000235:genetics; D002756:Cholagogues and Choleretics / Q000008:administration & dosage / Q000627:therapeutic use*; D004195:Disease Models, Animal; D004596:Electroretinography / Q000187:drug effects; D007279:Injections, Subcutaneous; D051379:Mice; D008869:Microtubule-Associated Proteins / Q000235:genetics; D009765:Obesity / Q000517:prevention & control*; D012160:Retina / Q000503:physiopathology; D012162:Retinal Degeneration / Q000188:drug therapy / Q000503:physiopathology; D012174:Retinitis Pigmentosa / Q000188:drug therapy* / Q000503:physiopathology; D013655:Taurochenodeoxycholic Acid / Q000008:administration & dosage / Q000627:therapeutic use*; D041623:Tomography, Optical Coherence
chemical_list: C506280:Bbs1 protein, mouse; D002756:Cholagogues and Choleretics; D008869:Microtubule-Associated Proteins; D013655:Taurochenodeoxycholic Acid; C031655:ursodoxicoltaurine