Although this is a less common ep form of the disease causes the most damage. Cat owners are forced

Glycogen storage disease type IV (Glycogen storage disease type IV - GSD IV) of the Norwegian forest cat was first described in 1988 in the United States by Professor John C. Fyfe. Outbreaks in Germany ep and France show that this problem is burdened not only cost the U.S. but also Europe. The results of genetic ep tests carried out in laboratories Laboklin and Antagene confirmed these concerns.
GSD IV is inherited abnormality of glucose metabolism. Under physiological conditions the excess ep of glucose, resulting in the process of metabolism of carbohydrates, proteins and fats is stored as glycogen in many different tissues, mainly in the liver and muscles. Glycogen molecules are highly branched structure, consisting of short chains of up 12-18 glucose residues. It allows deposition or release of glucose ep from glycogen, for example in a situation of increased energy demand.
The mutation ep results in abnormal expression of the gene encoding the amyloid-1 ,4-1 ,6-transglucosidase - glycogen branching enzyme (GBE) that is an enzyme of glycogen synthesis necessary to produce a branched molecular structure. GBE deficiency leads to pathological accumulation of glycogen in the cytoplasm of muscle cells, hepatocytes (liver cells) and neurons, causing a progressive impairment of the function of various organs, a slight to significant, potentiated. Inheritance GSD IV
GSD IV is inherited as an autosomal recessive trait. This means that it can be passed down from generation to generation unnoticed, until finally the combination of two cat carriers will be born sick kitten. However, if the disease is not properly diagnosed, cat carriers are not excluded from the mutation breeding program and will continue to be distributed directly to the population. ep
There are three variants of genotype: homozygous healthy ep (N / N) - the cat is free of the defective gene and the mutation does not transmit to offspring. homozygous affected (GSD IV / GSD IV) - cat sick. They will always pass the mutated gene to offspring probably did not survive to reproductive age. heterozygote - carrier ep (N / GSD IV) - has one copy of the mutated gene GBE 1 and one copy of the mutation-free. Not develop in his illness, but can pass the mutation offspring. After crossing two heterozygotes 25% kitten is sick, and 50% will be carriers. The same carriers born after combined heterozygous and homozygous healthy. ep Crossword carrier of the individual free of mutations and other Homozygous healthy heterozygote carrier - the carrier of N / NN / GSD IV heterozygote - Bearer N / GSD IV N / N - 50% N / N - 25% N / GSD IV - 50% N / GSD IV - IV 50% GSD / GSD IV - 25% of the clinical symptoms
In rare cases, a sick cat can survive the neonatal period and develop normally until the age of 5 months, when the onset of progressive degeneration of the nerve-muscle tremor of the head, ataxia, muscular dystrophy ep and heart failure, and enlargement of the spleen and liver cirrhosis , ascites. Convulsions may occur and high temperature, which is not relieved by corticosteroids. ep
Although this is a less common ep form of the disease causes the most damage. Cat owners are forced to helplessly watch the suffering ep of his ward. The cat has difficulty moving, eating alone and requires a lot of care. A few months later, in the final stage of the disease, died of multiple organ failure due to the body. Recognition
GSD IV, resulting in the death of kittens at birth may be confused with izoerytrolizą resulting from incompatibility of blood groups kittens and their mother. Symptoms of nerve other forms of GSD IV should be differentiated from cerebellar hypoplasia after meningitis and brain, and by FIP and toxoplasmosis.
In order to confirm the diagnosis can be performed histopathological examination, but only a diagnosis and DNA testing, allowing directly demonstrate the presence of a mutation responsible for the disease. Opportunities to combat ep the disease
At present ep there is no cure for the sick kittens. The progress of the disease seems to be inevitable and always fatal. Growers can not completely ep prevent przychodzeniu the world sick cats.
According to data from the laboratory Laboklin in 2008 9% of respondents cats are carriers of the mutation. If cats mating occurred at random, approximately 2-3 kittens in 1000 rodziłyby a chore. ep This did not happen, because the selection of breeding cats is not left to chance.
Breeders have a tool in the form of a genetic test that can not only distinguish cats suffering from the disease-free individuals from a mutation, ep but also the identification of clinically healthy carriers. To check the occurrence