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Cited by 115

The DOI matches its Crossref record; 4 of the 4 references it cites were found in a registry or an index.

Cholesterol biosynthesis pathway is disturbed in YAC128 mice and is modulated by huntingtin mutation

Valenza, M., Carroll, J. B., Leoni, V., Bertram, L. N., Bjorkhem, I., Singaraja, R. R., Di Donato, S., Lutjohann, D., Hayden, M. R., Cattaneo, E.·2007·Human Molecular Genetics
DOI 10.1093/hmg/ddm170
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Abstract

Our recent analyses of the cholesterol biosynthetic pathway in Huntington's disease (HD) cells, in the R6/2 huntingtin-fragment mouse model of HD as well as in human tissues have provided the first evidence of altered activity of this pathway in genetically identifiable HD samples. Here we report that these changes also occur in the full-length-huntingtin YAC128 (yeast artificial chromosome) mouse model, which shows a consistent reduction in the activity or levels of multiple components of the cholesterogenic pathway. We also show that this phenotype is progressive and is specific for the brain region most affected in HD. Mice over-expressing the wild-type protein with 18 CAG (YAC18 mice) show the opposite phenotype with higher activity of the cholesterol biosynthetic pathway compared with littermate mice. Finally, we report that plasma levels of cholesterol, its precursors and its brain-derived catabolite 24-S-hydroxycholesterol in YAC mice mirror brain biosynthetic levels supporting further investigation of their potential as peripheral biomarkers in HD.

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Crossref · 10.1093/hmg/ddm170

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4 of 4 references verified0 citation errors · 0 not checkable

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  • Cholesterol biosynthesis is severely reduced in the brain of HD mouse models, with the striatum affected first.

    “A large number of studies conducted in different HD rodent models have demonstrated significantly reduced biosynthesis of cholesterol in the brain, with the striatum being affected first 17,18,19,20,21 .”

    From SREBP2 gene therapy targeting striatal astrocytes ameliorates Huntington’s disease phenotypes (2021) · cites this paper as [19]

  • Steady-state levels of lanosterol, lathosterol, and desmosterol were reduced in the striata of R6/2-Tom mice compared with wt-Tom mice.

    “As expected, the steady-state levels of all cholesterol precursors were reduced in the striata of R6/2-Tom mice compared with wt-Tom mice (Fig. 2H-J) 19,20,27,28 .”

    From SREBP2 gene therapy targeting striatal astrocytes ameliorates Huntington’s disease phenotypes (2021) · cites this paper as [19]

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Metadata

Authors
Valenza, M., Carroll, J. B., Leoni, V., Bertram, L. N., Bjorkhem, I., Singaraja, R. R., Di Donato, S., Lutjohann, D., Hayden, M. R., & Cattaneo, E.
Venue
Human Molecular Genetics
Publisher
Oxford University Press (OUP)
Published
2007-06-27
Volume
16
Issue
18
Pages
2187-2198
ISSN
  • 0964-6906
  • 1460-2083
DOI
10.1093/hmg/ddm170
Cited by
115
References
4
Language
en

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