Abstract
Selective neuronal lipidosis and neuroaxonal dystrophy of the dorsal nucleus of Clarke and lateral cuneate nucleus were the neuropathological findings in 3 males with Zellweger syndrome examined at the Medical Unit S Carolina, Charleston, SC, the John F.
Highlights
The lacking peroxisomes are cytoplasmic organelles containing oxidases and catalase and are involved in metabolism of hydrogen peroxide, fatty acids and bile acids
Selective neuronal lipidosis and neuroaxonal dystrophy of the dorsal nucleus of Clarke and lateral cuneate nucleus were the neuropathological findings in 3 males with Zellweger syndrome examined at the Medical Unit S Carolina, Charleston, SC, the John F
Large amounts of abnormal cholesterol esters containing saturated and monosaturated very long-chain fatty acids were demonstrated in the striated neurons of the dorsal thoracic cord
Summary
The lacking peroxisomes are cytoplasmic organelles containing oxidases and catalase and are involved in metabolism of hydrogen peroxide, fatty acids and bile acids. Selective neuronal lipidosis and neuroaxonal dystrophy of the dorsal nucleus of Clarke and lateral cuneate nucleus were the neuropathological findings in 3 males with Zellweger syndrome examined at the Medical Unit S Carolina, Charleston, SC, the John F. Large amounts of abnormal cholesterol esters containing saturated and monosaturated very long-chain fatty acids were demonstrated in the striated neurons of the dorsal thoracic cord.
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