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Infantile neuronal ceroid lipofuscinosis

Isolation of storage material

Departments of Neurology (Drs. Palo and Elovaara) and Pathology (Dr. Haltia), University of Helsinki, and the Donner Laboratory of Experimental Neurochemistry (Drs. Kin and Wolfe), Montreal Neurological Institute and Hospital, McGill University, Montreal.

We used ultracentrifugation to isolate autofluorescent storage particles from the brains of patients with infantile type of neuronal ceroid lipofuscinosis (Haltia-Santavuori). The particles differed from those of the late infantile (Jansky-Bielschowsky) and juvenile (Batten-Spielmeyer-Sjögren) types and retained the characteristic appearance—large, partly membrane-bound conglomerates of spherical globules with a uniform finely granular internal structure—despite the vigorous procedure. The sedimentation properties of the particles also differed from the two other forms, but dolichols are found in the particles of all three diseases.

Address correspondence and reprint requests to Dr. Palo, Department of Neurology, University of Helsinki, Haartmaninkatu 4, 00290 Helsinki 29, Finland.

Supported by the Rinnekoti Research Institute, Espoo, Finland, and by the Finnish National Medical Research Council.

Accepted for publication February 22, 1982.

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