Progress toward positional cloning of ovine neuronal ceroid lipofuscinosis, a model of the human late-infantile variant CLN6

The neuronal ceroid lipofuscinoses (NCLs) are lysosomal storage diseases wi th severe neurodegenerative pathology. An ovine model (OCL) has well-define d parallels with the human disease at the biochemical and pathological leve ls. The gene for OCL is located in the chromosomal region OAR7q13-15. This region is syntenic with HAS15q21-23, suggesting that OCL and CLN6 represent mutations in orthologous genes. New microsatellite markers have been devel oped enabling further refinement of the OCL critical region. (C) 1999 Acade mic Press.