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Dog hereditary diseases

DSD021
Neuronal ceroid lipofuscinosis type 4A
NCL IVA
5
Dogs
Cerebral Ataxia, Neuronal Ceroid Lipofuscinosis, NCL-A
Hereditary diseases

Description
Cerebellar ataxia in American Staffordshire Terrier (AST) is an inherited autosomal recessive neurological disease caused by lysosomal disorders, followed by degeneration of the cerebellum. Cerebellar ataxia AST belongs to a family of diseases called Neuronal ceroid lipofuscinose (NCL), which is why NCL-A is used to denote the disease.

Symptoms
unsteady gait, falling on the side of the dog, difficulty turning, falling from shaking his head, tremors. To maintain balance, the dog begins to spread its legs wide. The disease is progressive, no treatment has been developed. The first signs usually appear between the ages of three and five.

Diagnostics
For the diagnosis of NCL-A, a genetic test has been developed that can be performed at any age. A DNA test detects a defective (mutant) copy of a gene and a normal copy of a gene. The test result is the definition of the genotype, according to which animals can be divided into three groups: healthy (homozygotes for the normal copy of the gene, NN), carriers (heterozygotes, NM), and patients (homozygotes for the mutation, MM).

  • Autosomal recessive inheritance

    AR
    MM - sick
    NM - healthy, carrier
    NN is healthy
ARSG
  • American bully
  • American pit bull terrier
  • American staffordshire terrier

Individual breeds