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The origin of renal galactosylceramide in the twitcher mouse, an animal model of human globoid cell leukodystrophy



The origin of renal galactosylceramide in the twitcher mouse, an animal model of human globoid cell leukodystrophy



Fukuoka Igaku Zasshi 79(7): 529-536



In order to elucidate the origin of the accumulating galactosylceramide in the twitcher mouse kidney, glycosphingolipids in the brain and kidney of the twitcher mouse were analyzed after intraperitoneal injection of radioactive galactose, and the following results were obtained. 1) In the kidney, there was no difference of glucosylceramide contents among twitcher, heterozygote and normal mice at 19 and 36 days postnatal, whereas three-fold increase of non-hydroxy fatty acid-galactosylceramide (NFA-gal-cer) was observed in the twitcher mouse at 36 days postnatal. The amount of .alpha.-hydroxy fatty acid-galactosylceramide (HFA-gal-cer) in the twitcher mouse was about ten and 15 times of values of heterozygote and normal mice, at 19 days and 36 days postnatal, respectively. 2) After intraperitoneal injection of [14C] galactose into normal mouse, the radioactivities in the Folch's upper phase solids, total lipids and chloroform-methanol insoluble residue of brain and kidney reached the peak within 24 hours and steadily declined thereafter. 3) Both total radioactivity and relative radioactivity (total radioactivity/weight) of HFA-gal-cer in heterozygote and normal mouse kidney decreased 17 days after intraperitoneal injection of [3H] galactose, while those in the twitcher mouse kidney increased in this period; the total radioactivity increased to 4-fold and the relative radioactivity to about 1.5-fold, compared to the value 24 hours after injection. In contrast to kidney, the total radioactivities in the brain of twitcher, heterozygote and normal mice increased about 1.4-fold, while relative radioactivity decreased; heterozygote and normal mice to less than half, and twitcher mice to 70% of the value of 24 hours after injection. From these results, the increase of HFA-gal-cer radioactivities in the twitcher mouse kidney seems to be not due to de novo synthesis but due to be transported from other tissues, because the half-life of the radioactive precursor of the lipid was less than 24 hours. Quantitatively, galactosylceramide is exclusively localized in the nervous tissue. Therefore, most of the accumulated HFA-gal-cer in the kidney is probably derived from the nervous tissue.

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Accession: 007920884

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PMID: 3235022



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