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December 1967

Inositide Metabolism in Rabbit Hereditary Ataxia

Author Affiliations

St. Louis; Bar Harbor, Me
From the Department of Neurology, Washington University School of Medicine, St. Louis (Dr. Eliasson and Mrs. Scarpellini) and The Jackson Laboratory, Bar Harbor, Me (Dr. Fox).

Arch Neurol. 1967;17(6):661-665. doi:10.1001/archneur.1967.00470300103017

HEREDITARY ataxia in rabbits is due to a recessive mutant gene (ax). The pathology begins in adolescence and progressively involves lateral brainstem nuclei and deep cerebellar nuclei. The clinical and morphological features have been described by Sawin et al1 and by O'Leary and his associates.2 Glycogen deposits in the neuropil of the involved areas have been demonstrated3 and a study of brain glycogen metabolism has been published by Tourtellotte and his co-workers.4 Glucose-6-phosphate was found to be significantly elevated with other intermediates at normal levels. Normal also were the amino acid spectra of brain and urine from ataxic rabbits as determined by Weinstein et al.5

Further biochemical studies were directed toward the lipid composition of the brainstem areas with histological evidence of lesions. A decrease in the concentration of inositolphosphatides was found6 and the present communication is an extension of these observations with

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