Dolichols in Brain and Urinary Sediment in Neuronal Ceroid Lipofuscinosis

Overview

Journal Neurology

Specialty Neurology

Date 1983 Jan 1

PMID 6681549

Citations 13

Authors

L S Wolfe

N M Ng Ying Kin

J Palo

M Haltia

Affiliations

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Abstract

Long-chain polyisoprenoid alcohols (dolichols) increase more than tenfold from age 5 to 80 years in human cerebral cortex. The dolichol content of brain from infantile, late infantile, and juvenile forms of neuronal ceroid lipofuscinosis (NCL) was significantly higher than in age-matched patients with other neurologic diseases. Significant increase of dolichols was also found in the urinary sediment in all three types of NCL patients, and this test is useful in making the diagnosis. Dolichol accumulation is the first biochemical marker of NCL and seems to parallel storage of ceroid lipofuscin.

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