Retinal pathology in the PPCD1 mouse

Abstract

Retinal phenotypes of the PPCD1 mouse, a mouse model of posterior polymorphous corneal dystrophy, have been characterized. PPCD1 mice on the DBA/2J background (D2.Ppcd1) have previously been reported to develop an enlarged anterior chamber due to epithelialization and proliferation of the corneal endothelium and subsequent blockage of the iridocorneal angle. Results presented here show that D2.Ppcd1 mice develop increased intraocular pressure (IOP), with measurements at three months of age revealing significant increases in IOP. Significant retinal ganglion cell layer cell loss is observed at five months of age. D2.Ppcd1 animals also exhibit marked degeneration of the outer nuclear layer in association with hyperplasia of the retinal pigment epithelium. Evidence of retinal detachment is present as early as three weeks of age. By 3.5 months of age, focal areas of outer nuclear layer loss are observed. Although the GpnmbR150X mutation leads to increased IOP and glaucoma in DBA/2J mice, development of anterior segment and retinal defects in D2.Ppcd1 animals does not depend upon presence of the GpnmbR150X mutation.

Corneal Dystrophy in Dutch Belted Rabbits as a Possible Model of Thiel-Behnke Subtype of Epithelial-Stromal TGFβ-Induced Corneal Dystrophy

“…37 The PPCD1 mouse is an accidental gene targeting mutation of Zeb -related pathways, with features similar to the ovo-like zinc finger 2 gene defect associated with human PPCD1, but these mice also have iridocorneal endothelial syndrome with retinal detachment. 62,63 Heritable late-onset FECD-like disease has been phenotypically described in German shorthaired and wirehaired pointers 49 and Boston terriers 52 but not genotyped. Mice deficient in WW domain-containing transcription regulator 1 have a preliminary phenotype of late-onset FECD including decreased endothelial cells and altered Descemet membrane.…”

“…44 Stromal dystrophies were found in an induced mutation in a mouse 32 and spontaneously in several breeds of dogs, [45][46][47][48]50 including a genotypically verified missense mutation that results in macular corneal dystrophy in Labrador retrievers. 51 Numerous induced mutations in mice have been created as models of corneal endothelial dystrophies, 33,35,[37][38][39]42,62,63 and spontaneous endothelium dystrophies have occurred in dogs. 49,52 Although mice with targeted mutations for genes linked to corneal dystrophy subtypes in humans provide strong evidence for their use as animal models of human disease, the available phenotypic characterization of these mice models is weak, and the biological activity of the gene defects, particularly for phenotypic-genotypic relationships of endothelial corneal dystrophies, has not been confirmed to be totally comparable across species.…”

Processes in Doping System: Quantification Reports in Mixed Martial Arts Fighters

“…It is important to see that the doping ratio has decreased from 2015 to 2022, which shows that the UFC USADA program has been effective. Regarding the top-ranked fighters tested with no anti-doping violations, it is important to note that the list includes several world champions such as Jose Aldo [46], Amanda Nunes [47], Kamaru Usman [48], Dustin Poirier [49], Rafael Dos Anjos [50], Holly Holm [51], Stipe Miocic [52], Conor McGregor [53], Daniel Cormier [54], Rose Namajunas [55], Francis Ngannou [56], Dominik Cruz [57], Israel Adesanya [58], Glover Teixeira [59], Robbie Lawler [60], Max Holloway [61], Aljamain Sterling [62], Cody Garbrandt [63], Valentina Shevchenko [64], Chris Weidman [65], Jiri Prochazka [66], Henry Cejudo [67], Andrei Aslovski [68], Carla Esparza [69], Khabib Nurmagomedov [70], Joanna Jedrzejczyk [71], Tyron Woodley [72], Deiveson Alcantara Figueiredo [73], Julianna Pena [74] and Jan Blachowicz [75].…”