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Trapped Neutrophil Syndrome in a Border Collie Dogs
Brunclíková, Tereza ; Krejčířová, Romana (advisor) ; Chmelíková, Eva (referee)
Border Collie comes from the part of the United Kingdom which is called Border Country. It's a herding dog, which should be obedient, smart, lively, and attentive. For breeding, Border Collies must fulfil bonitation conditions. One of the mandatory tests is the test on hereditary neutropenia. Hereditary neutropenia is a disease which causes a fatal immune system failure.
Immunity; or defences; is one of the basic features for survival. Basic ability of cells of the immune system is to recognize when in contact with other molecules the structure is inherent or not. Memory, which is another ability of the immune system, ensures a prompter, more intensive, and quicker response when meets already identified antigen repetitively.
Neutrophils are granulocytes (a type of white blood cells), which contain granules. These granules have active substances which participate in inflammation and allergic reactions. Neutrophils are produced in the bone marrow. They are brought by bloodstream to the site of inflammation where they phagocytose bacteria.
Hereditary neutropenia (Trapped Neurophil Syndrome - TNS) is a hereditary autosomal recessive disease. Hereditary disease is transferred from parents to offspring. In an autosomal recessive disease a feature is transferred by a recessive allele. A monitored feature is phenotypically shown just with recessive homozygotes. Heterozygotes are with no clinical signs of the disease but they cannot be phenotypically distinguished from dominant homozygotes.
TNS is characterized by a significant lack of neutrophils in blood because they are not brought from the bone marrow into the blood circulation. This is caused by retention at the site of their origin - in the bone marrow.
It is assumed that all cases of TNS are derived from one ancestor because in the pedigrees they have the same ancestor in the past six generations.
Development of an affected dog is generally slowed down. Puppies have smaller size; and for TNS disease is typical a shape of facial skull which resembles a ferret. The individuals suffer from fever and swollen joints. Affected dogs are dying at an early age because of immune system failure.
It was found that hereditary neutropenia is very similar to Cohen syndrome, which is a human disease. Both diseases have similar symptoms; it has been proved that both are caused by mutation of the same gene.
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