Neuronal ceroid lipofuscinosis nclf

Neuronal ceroid lipofuscinosis (nclf )

nclf/nclf mice develop progressive ataxia defects, myelination defects, neurodegeneration, neuromuscular defects and retinal degeneration. In homozygous nclf mice, the outer nuclear layer shows cell loss by four months after birth. By 6 months the peripheral outer retina is more severely affected, and by 9 months the entire retina is atrophied.