Neuronal Ceroid Lipofusinosis (CL)

Neuronal Ceroid Lipofuscinosis is a type of lysosomal storage disorder that results in accumulation of lysosomal storage bodies in the cells of many tissues of the affected animal. This leads to progressive and severe neurological impairment and early death. Affected dogs appear normal at birth, but begin to exhibit symptoms early in life – around 1- 2 years of age. The age of onset and severity of the disease can vary greatly among individuals. The symptoms include progressive motor decline with seizures and loss of coordinated muscle movements, cognitive decline and abnormal behavior. Visual impair. The mutation causing the form of disease found in Border Collies was identified by Scott Melville in the laboratory of Dr. Alan Wilton of the school of Biotechnology of Biomolecular sciences at the University of NSW.  There is no treatment and no cure, but a DNA test is now available to detect carriers as well as affected dogs.  All reputable breeders in Australia would have their breeding stock DNA tested before producing a litter.