June 2013
Volume 54, Issue 15
Free
ARVO Annual Meeting Abstract  |   June 2013
ZONULAR LIGAMENT DYSPLASIA IN BEAGLES WITH HEREDITARY PRIMARY OPEN ANGLE GLAUCOMA (POAG)
Author Affiliations & Notes
  • Leandro Teixeira
    Pathobiological Science, UW-Madison Sch of Vet Med, Madison, WI
  • Erin Scott
    Pathobiological Science, UW-Madison Sch of Vet Med, Madison, WI
  • Simone Iwabe
    School of Veterinary Medicine, University of Pennsylvania, Philadelphia, PA
  • Richard Dubielzig
    Pathobiological Science, UW-Madison Sch of Vet Med, Madison, WI
  • Andras Komaromy
    College of Veterinary Medicine, Michigan State University, East Lansing, MI
    School of Veterinary Medicine, University of Pennsylvania, Philadelphia, PA
  • Footnotes
    Commercial Relationships Leandro Teixeira, None; Erin Scott, None; Simone Iwabe, None; Richard Dubielzig, OSOD, LLC (I), Allergan (C); Andras Komaromy, None
  • Footnotes
    Support None
Investigative Ophthalmology & Visual Science June 2013, Vol.54, 3564. doi:https://doi.org/
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      Leandro Teixeira, Erin Scott, Simone Iwabe, Richard Dubielzig, Andras Komaromy; ZONULAR LIGAMENT DYSPLASIA IN BEAGLES WITH HEREDITARY PRIMARY OPEN ANGLE GLAUCOMA (POAG). Invest. Ophthalmol. Vis. Sci. 2013;54(15):3564. doi: https://doi.org/.

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

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Abstract
 
Purpose
 

To characterize the microscopic and ultrastructural morphology of the anterior uvea of Beagles with autosomal recessively inherited POAG due to a G661R missense mutation in the ADAMTS10 gene.

 
Methods
 

Four animals were analyzed, a 6-month old (6m-/+) and a 3-year old (3y-/+) non-affected dog heterozygous for ADAMTS10 mutation, and two age-matched affected dogs homozygous for the mutation (6m-/-; 3y-/-G); the older affected dog showed clinical signs of glaucoma. Animals were enucleated and globes processed for histology and transmission electron microscopy (TEM).

 
Results
 

Grossly the 3y-/-G animal presented markedly irregular ciliary processes. Both homozygous animals presented with thick and homogenous eosinophilic, PAS-positive and Masson’s trichrome blue, proteinaceous material carpeting the ciliary body surface, consistent with collapsed dysplastic zonular ligaments. TEM showed a thick mat of dysplastic zonular ligament fibrils associated with markedly irregular and reduplicated basement membranes carpeting the surface of the ciliary body epithelium. The 3y-/-G animal also presented moderate disruption of the collagen fibers in the core of the trabecular meshwork beams. All animals presented normal scleral collagen.

 
Conclusions
 

The lesions are consistent with previously described zonular ligament dysplasia of terrier dog breeds affected by a mutation in ADAMTS17. Mutations in both ADAMTS10 and ADAMTS17 are responsible human Weill-Marchesani syndrome. The zonular and TM collagen lesions suggest the mutation impacts the metabolism of these proteins in the ocular tissues. Histological and TEM features of the trabecular meshwork are compatible with POAG and support the use of these animals as POAG model.

 
 
Fig. 1. Ciliary epithelium. A. 6m-/+ B. 3y-/+ Normal ciliary epithelium and zonules protein carpeting the epithelium surface. C. 6m-/- and D. 3y-/-G. Homogenous, multi-laminated and eosinophilic proteinaceous material carpeting the ciliary body epithelium interpreted as collapsed dysplastic zonules. Alcian-blue PAS.
 
Fig. 1. Ciliary epithelium. A. 6m-/+ B. 3y-/+ Normal ciliary epithelium and zonules protein carpeting the epithelium surface. C. 6m-/- and D. 3y-/-G. Homogenous, multi-laminated and eosinophilic proteinaceous material carpeting the ciliary body epithelium interpreted as collapsed dysplastic zonules. Alcian-blue PAS.
 
 
Fig. 2. TEM. A. 6m-/+ and B. 3y-/+. Normal epithelial cells with associated zonules and zonular fibers (2B inset). C. 6m-/- and D. 3y-/-G. Epithelial cells with markedly duplicated basement membranes (arrow) and thick mat of dysplastic zonular fibrils carpeting the cell surface(* and 2D inset).
 
Fig. 2. TEM. A. 6m-/+ and B. 3y-/+. Normal epithelial cells with associated zonules and zonular fibers (2B inset). C. 6m-/- and D. 3y-/-G. Epithelial cells with markedly duplicated basement membranes (arrow) and thick mat of dysplastic zonular fibrils carpeting the cell surface(* and 2D inset).
 
Keywords: 421 anterior segment • 455 ciliary body • 637 pathology: experimental  
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