Primary Lens Luxation (PLL)



Primary Lens Luxation

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Breeds: Alapaha Blue Blood Bulldog, American Corgi, American Hairless Terrier, American Hunt Terrier, American Morkshire Terrier, Australian Cattle Dog, Australian Kelpie, Australian Stumpy Tail Cattle Dog, Australian Terrier, Berger Blanc Suisse, Biewer Terrier, Bouvier des Flandres, Bull Terrier, Cardigan Welsh Corgi, Cesky Terrier, Chinese Crested, Danish Swedish Farmdog, Fox Terrier, German Hunter Terrier, German Shepherd Dog, Jack Russell Terrier, Jagd Terrier, Japanese Chin, Lakeland Terrier, Lancashire Heeler, Miniature Bull Terrier, Mixed Breed, Norfolk Terrier, Norwich Terrier, Parson Russell Terrier, Portuguese Podengo Pequeno, Pug, Puli, Pyrenean Shepherd, Rat Terrier, Russell Terrier, Scottish Terrier, Sealyham Terrier, Silky Terrier, Smooth Fox Terrier, Soft Coated Wheaten Terrier, Swiss White Shepherd, Teddy Roosevelt Terrier, Tibetan Terrier, Toy Fox Terrier, Unspecified, Welsh Terrier, Whoodle, Wire Fox Terrier, Yorkiepoo, Yorkshire Terrier

Description

Primary Lens Luxation (PLL) is a disorder that is characterized by weakened zonular fibers which eventually lead to the dislocation of the lens in the eye. In the eye of a canine, the lens is located directly behind the iris and pupil. It is responsible for focusing light to the retina in the back of the eye. The zonular fibers are responsible for holding the lens in place.

In dogs affected by PLL, these fibers are much weaker and can the lens can dislocate or luxate. This is very painful for the dog. Depending on where the lens moves to, it can leave the dog permanently blind. Often, this event will occur around 3-6 years of age. It is recommended that all dogs genetically affected by PLL be evaluated by a veterinarian every 6 months to monitor the condition of the eyes.

Primary Lens Luxation is generally an autosomal recessive disorder. Autosomal recessive disorders are disorders that can be passed from either parent and require two copies of the gene to show symptoms. However, there have been cases of a carrier dog having a luxated lens. Carriers are dogs that have one copy of the mutation responsible for PLL, and can pass that mutation on to any offspring. Typically carriers have a lesser chance of the lens luxating. It is important to test dogs to see if they carry for the PLL disorder. If two carriers are bred together, there is a 25% chance per puppy that they will develop the PLL disorder and have a higher chance of the lens luxating.

Not all cases of a luxated lens are from Primary Lens Luxation. Dogs that are genetically clear of the mutation could potentially experience this event at some point. This could be from an injury, other eye disorders, or an infection.

Possible Results

Genotype Description
PLL/PLL Affected: Dog has two copies of the PLL mutation and will be affected. The gene will be passed on to every offspring.
n/PLL Low Risk: Dog has one copy of the PLL mutation. Dog has a slight risk of developing PLL and may pass the mutation on to offspring.
n/n Clear: Dog is negative for mutation associated with PLL.

Reference

Farias FH, Johnson GS, Taylor JF, Giuliano E, Katz ML, Sanders DN, Schnabel RD, McKay SD, Khan S, Gharahkhani P, O'Leary CA, Pettitt L, Forman OP, Boursnell M, McLaughlin B, Ahonen S, Lohi H, Hernandez-Merino E, Gould DJ, Sargan DR, Mellersh C. An ADAMTS17 splice donor site mutation in dogs with primary lens luxation. Invest Ophthalmol Vis Sci. 2010 Sep; 51(9):4716-21. [PubMed: 20375329]