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NCL

The Neuronal ceroid lipofuscinosis (NCLs) are a group of inherited neurodegenerative diseases characterized by accumulation of autofluorescent cytoplasmic antibodies within cells of the nervous system.

A young-adult onset NCL occurs in American Bulldogs and a mutation in the cathepsin D gene (CSTD) responsible for NCL in this breed has been identified (Awano et al 2006).

NCL is inherited as an autosomal recessive trait. This means that affected animals have two copies of the mutant gene and will develop lethal NCL. Affected dogs typically exhibit a condition of cerebellar dysfunction in which voluntary muscular movements tend to result in the movement of bodily parts beyond the intended goal and the loss of the ability to coordinate muscular movement. American bulldogs that have one mutant and one normal gene are called carriers. Carriers are not ill, but can produce affected offspring if mated to another carrier.