+ Site Statistics
+ Search Articles
+ Subscribe to Site Feeds
EurekaMag Most Shared ContentMost Shared
EurekaMag PDF Full Text ContentPDF Full Text
+ PDF Full Text
Request PDF Full TextRequest PDF Full Text
+ Follow Us
Follow on FacebookFollow on Facebook
Follow on TwitterFollow on Twitter
Follow on LinkedInFollow on LinkedIn

+ Translate

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.

(PDF emailed within 1 workday: $29.90)

Accession: 007920884

Download citation: RISBibTeXText

PMID: 3235022

Related references

Metabolism of galactosylceramide in the twitcher mouse an animal model of human globoid cell leukodystrophy. Biochimica et Biophysica Acta 879(2): 215-220, 1986

The twitcher mouse A model of human globoid cell leukodystrophy. Martenson, R E Myelin: Biology and chemistry ix+958p CRC Press, Inc : Boca Raton, Florida, USA; London, England, UK Illus ISBN 0-8493-8849-X : 745-759, 1992

The twitcher mouse. A model of human globoid cell leukodystrophy (krabbe's disease). American Journal of Pathology 111(3): 394-397, 1983

The Twitcher mouse: an enzymatically authentic model of human globoid cell leukodystrophy (Krabbe disease). Brain Research 202(2): 479-483, 1980

Hematopoietic cell transplantation hct in murine globoid cell leukodystrophy gcl the twitcher mouse a model of human sphingolipid storage krabbe disease. Experimental Hematology (Charlottesville) 15(5): 449, 1987

Globoid cell leukodystrophy: specialized contact of globoid cell with astrocyte in the brain of twitcher mouse. Acta Neuropathologica 58(4): 237-242, 1982

Genetic background markedly influences vulnerability of the hippocampal neuronal organization in the "twitcher" mouse model of globoid cell leukodystrophy. Journal of Neuroscience Research 77(4): 507-516, 2004

Suppression of galactosylceramidase (GALC) expression in the twitcher mouse model of globoid cell leukodystrophy (GLD) is caused by nonsense-mediated mRNA decay (NMD). Neurobiology of Disease 23(2): 273-280, 2006

Severe graft versus host disease in a patient with globoid cell leukodystrophy following umbilical cord blood transplantation: resemblance to the twitcher mouse model. Turkish Journal of Pediatrics 49(3): 304-306, 2007

An accumulation of galactocerebroside in kidney from mouse globoid cell leukodystrophy (twitcher). Biochemical and Biophysical Research Communications 109(3): 634-638, 1982

Fingerprint inclusions in kidney of mouse globoid cell leukodystrophy twitcher mouse. Jikeikai Medical Journal 31(2): 199-202, 1984

Demyelination in the spinal cord of murine globoid cell leukodystrophy (the twitcher mouse). Acta Neuropathologica 62(4): 298-308, 1984

Nerve conduction studies in the Twitcher mouse (murine globoid cell leukodystrophy). Journal of the Neurological Sciences 74(2-3): 307-318, 1986

Sphingolipid profile in the CNS of the twitcher (globoid cell leukodystrophy) mouse: a lipidomics approach. Cellular and Molecular Biology 49(5): 779-787, 2003

Murine globoid cell leukodystrophy: the twitcher mouse. An ultrastructural study of the kidney. Laboratory Investigation; A Journal of Technical Methods and Pathology 50(1): 42-50, 1984