Why are Shar Pei dogs so wrinkly? Surprising Answer

Keeping Your Shar-Pei Ship Shape

The genetics of the Shar-Peis wrinkles helped Lindblad-Toh and other researchers uncover a mystery when they used the breed as subjects in a 2011 study connecting Shar-Pei to a fairly common canine illness.

The study linked the HAS2 gene with a tendency of Shar-Pei to fall ill with periodic fever disease, sometimes referred to as Familial Shar-Pei Fever (FSF). Symptoms include episodes of fever that appear to rise out of nowhere, and overall listlessness. Lindblad-Toh says hyaluronan is to blame for periodic fever disease in Shar-Pei and the teams research bears it out.

Why are Shar Pei dogs so wrinkly?

First, they compared the Shar-Pei genome with other dog breeds, then compared it to healthy and sick Shar-Pei, searching for the mutation to the fever. And each time they came to the same conclusion. “In the study, a mutation (in a strand of DNA) affecting both the fever syndrome and the wrinkled skin was found,” says Lindblad-Toh. “The duplication mutation was seen near the HAS2 gene, more copies the mutation was associated with and more HAS2, as well as the periodic fever syndrome.”

What does it all mean? In addition to being genetically predisposed to periodic fever disease, Shar-Pei are also at risk for Shar-Pei Autoinflammatory Diseases (SPAID) says Lindblad-Toh. “Several symptoms are present,” she says, “including recurrent fever, arthritis, dermatitis, otitis (ear infection) and systemic reactive amyloidosis (an inflammatory disease in the bodys organs).” Fortunately, Shar-Pei can be tested for SPAID to help owners make better informed breeding decisions.

There are other health issues that can be challenging to Shar-Pei. Because of the folds in Shar-Pei skin, they are susceptible to certain skin problems and a serious eye disorder called entropion, when the eyelid (usually the lower lid) folds inward, causing irritation to the cornea. Other eye problems of concern to the breed are glaucoma, pressure on the eye that can cause blindness; retinal dysplasia, an inherited disease that causes a malformation of the retina; “cherry eye,” a prolapsed gland in the third eyelid; chemosis, swelling of the lining of the eye — typical in puppies or young Shar-Pei; and Sudden Acquired Retinal Degeneration Syndrome or SARDS, a rare disease that causes blindness.

The Chinese Shar-Pei Club of America also recommends that owners have their dogs screened for a number of health issues including:

  • Hip dysplasia
  • Elbow dysplasia
  • Autoimmune thyroiditis
  • Patellar luxation (dislocation of the kneecap)
  • Canine Eye Registration Foundation (CERF) exam to detect eye and vision irregularities
  • A group of researchers at Universitat Autònoma de Barcelona (UAB) have discovered the origin of the mucinosis present in Shar Pei dogs, a hereditary disorder responsible for the characteristic wrinkles found in this breed.

    Materials provided by Universitat Autònoma de Barcelona. Note: Content may be edited for style and length.

    A group of researchers at UAB, led by professors Lluís Ferrer and Anna Bassols, have discovered the mechanisms of this disorder. Mucinosis in Shar Peis is due to an accumulation of hyaluronic acid (one of the main components found in the space between tissue cells) in cutaneous structures. This causes them to present high levels of hyaluronic acid in their bloodstream as well. Research with fibroblast cell cultures isolated from the Shar Peis skin have allowed scientists to demonstrate that synthesis of hyaluronic acid is produced in abnormally high amounts due to an excess in the activity (overexpression) of the HAS2 enzyme, one of the three enzymes responsible for the synthesis of acid in mammals. Now researchers are working to find the exact location of the genetic mutation causing this metabolic alteration, in a collaboration project between Armand Sánchez and Olga Francino from the UAB Department of Animal and Food Science, and Mia Olson and Kerstin Lindblah-Tod from the University of Uppsala, Sweden.

    The research was carried out by researchers Lluís Ferrer, Giordana Zanna, Dolors Fondevila, Anna Bassols and Maria José Docampo from the UAB Department of Animal Medicine and Surgery, and the Department of Biochemistry and Molecular Biology. The results have been published in the journals Veterinary Dermatology and Journal of Heredity and will be presented at the World Congress of Veterinary Dermatology (WCVD6) which will take place in Hong Kong on 19 November 2008.

    The research report appears in the journals Veterinary Dermatology and Journal of Heredity. The report details the genetic alteration in this breed which multiplies the activity of an enzyme responsible for an excessive production of hyaluronic acid, a substance which gathers under the skin and produces wrinkles. Understanding this molecular mechanism will give way to new reproduction programmes aimed at improving the health of these animals. It also will be used to learn more about human disorders such as Familial Mediterranean Fever (FMF) or mucinosis, and to gain more knowledge on the ageing process.

    The researchers found many genes that could have an influence on the size of the dog or the color of the coat, and they also identified specific differences in a gene that results in the wrinkled skin of the Shar-Pei. They made this identification by comparing the genes in 32 Shar-Peis with highly wrinkled skin to those of 18 Shar-Peis with smoother skin. Akey said he had decided to study the Shar-Pei particularly because there are rare mutations in humans that also produce severe wrinkling.

    The affected gene, HAS2 makes an enzyme (hyaluronic acid synthase 2) that is important in the production of skin tissue. Akey speculated that a mutation occurred and a breeder liked the look of the wrinkled puppy and selectively bred for the trait.

    The research, by Joshua Akey and colleagues from the University of Washington in Seattle in the US, sequenced large portions of the genes of 300 dogs of 10 pedigree breeds, including the Shar-Pei, Standard Poodle and Jack Russell. Their aim was to determine which areas were likely to have been involved in selective breeding and to identify the genes corresponding to selected physical features.

    Unlike previous research, which began with the traits and looked for corresponding genes, Akey and his colleagues started with the genes and looked for regions that were different in the various breeds, and then looked for physical attributes that might be related to the changes. They located 155 distinct genetic regions that appeared to have been tampered with through breeding, including five genes that have been linked previously to differences between breeds.

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