ALS ja endogeeninen kolesterolisynteesi

 HAKU: 6 vastausta, tuorein on preprint. 

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Showing results for als and endogen cholesterol synthesis

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1

Up-regulation of cholesterol synthesis pathways and limited neurodegeneration in a knock-in Sod1 mutant mouse model of ALS.

Dominov JA, Madigan LA, Whitt JP, Rademacher KL, Webster KM, Zhang H, Banno H, Tang S, Zhang Y, Wightman N, Shychuck EM, Page J, Weiss A, Kelly K, Kucukural A, Brodsky MH, Jaworski A, Fallon JR, Lipscombe D, Brown RH. bioRxiv. 2023 May 5:2023.05.05.539444. doi: 10.1101/2023.05.05.539444. Preprint. PMID: 37205335 Free PMC article.

By contrast, cholesterol synthesis genes are down-regulated in severely affected human TgSOD1 (G93A) transgenic mice at 4 months. Our analyses implicate dysregulation of cholesterol or related lipid pathway genes in ALS pathogenesis. The Sod1 (G85R) kn …

2

Regulation of Brain Cholesterol: What Role Do Liver X Receptors Play in Neurodegenerative Diseases?

Mouzat K, Chudinova A, Polge A, Kantar J, Camu W, Raoul C, Lumbroso S. Int J Mol Sci. 2019 Aug 8;20(16):3858. doi: 10.3390/ijms20163858. PMID: 31398791 Free PMC article. Review.

Liver X Receptors (LXR) alpha and beta are two members of nuclear receptor superfamily documented as endogenous cholesterol sensors. Following conversion of cholesterol in oxysterol, both LXR isoforms detect intracellular concentrations and act as transcripti …

3

Mitochondrial dysfunction and decrease in body weight of a transgenic knock-in mouse model for TDP-43.

Stribl C, Samara A, Trümbach D, Peis R, Neumann M, Fuchs H, Gailus-Durner V, Hrabě de Angelis M, Rathkolb B, Wolf E, Beckers J, Horsch M, Neff F, Kremmer E, Koob S, Reichert AS, Hans W, Rozman J, Klingenspor M, Aichler M, Walch AK, Becker L, Klopstock T, Glasl L, Hölter SM, Wurst W, Floss T. J Biol Chem. 2014 Apr 11;289(15):10769-10784. doi: 10.1074/jbc.M113.515940. Epub 2014 Feb 10. PMID: 24515116 Free PMC article.

The majority of amyotrophic lateral sclerosis (ALS) cases as well as many patients suffering from frontotemporal lobar dementia (FTLD) with ubiquitinated inclusion bodies show TDP-43 pathology, the protein encoded by the TAR DNA-binding protein (Tardbp) gene. We used recom …

4

Lipid oxidation and peroxidation in CNS health and disease: from molecular mechanisms to therapeutic opportunities.

Adibhatla RM, Hatcher JF. Antioxid Redox Signal. 2010 Jan;12(1):125-69. doi: 10.1089/ars.2009.2668. PMID: 19624272 Review.

Reactive oxygen species (ROS) are produced at low levels in mammalian cells by various metabolic processes, such as oxidative phosphorylation by the mitochondrial respiratory chain, NAD(P)H oxidases, and arachidonic acid oxidative metabolism. To maintain physiological redo …

5

T lymphocytes potentiate endogenous neuroprotective inflammation in a mouse model of ALS.

Chiu IM, Chen A, Zheng Y, Kosaras B, Tsiftsoglou SA, Vartanian TK, Brown RH Jr, Carroll MC. Proc Natl Acad Sci U S A. 2008 Nov 18;105(46):17913-8. doi: 10.1073/pnas.0804610105. Epub 2008 Nov 7. PMID: 18997009 Free PMC article.

Cell-specific flow cytometry and gene expression profiling showed significant phenotypic changes in microglia, including dendritic cell receptor acquisition, and expression of genes linked to neuroprotection, cholesterol metabolism and tissue remodeling. Microglia d …

6

Neuropathologic and biochemical changes during disease progression in liver X receptor beta-/- mice, a model of adult neuron disease.

Bigini P, Steffensen KR, Ferrario A, Diomede L, Ferrara G, Barbera S, Salzano S, Fumagalli E, Ghezzi P, Mennini T, Gustafsson JA. J Neuropathol Exp Neurol. 2010 Jun;69(6):593-605. doi: 10.1097/NEN.0b013e3181df20e1. PMID: 20467332

Alterations of brain cholesterol homeostasis have recently been considered as possible cofactors in many neurodegenerative disorders, including ALS. ...These results suggest that LXRbeta may inhibit neuroinflammation and maintain cholesterol homeostasis, and …