![[Valid RSS]](images/valid-rss-rogers.png "Valid RSS")
This is a valid RSS feed.
This feed is valid, but interoperability with the widest range of feed readers could be improved by implementing the following recommendations.
[help]
<rss version="2.0" xmlns:prism="http://purl.org/rss/1.0/modules/prism/">
line 17, column 6: (4 occurrences) [help]
<prism:startingPage xmlns:prism="prism">awaf328</prism:startingPage>
^
</channel>
^
<?xml version="1.0"?><rss version="2.0" xmlns:prism="http://purl.org/rss/1.0/modules/prism/"> <channel> <title>Brain Advance Access</title> <link>http://academic.oup.com/brain</link> <description> </description> <language>en-us</language> <pubDate>Tue, 16 Sep 2025 00:00:00 GMT</pubDate> <lastBuildDate>Mon, 15 Sep 2025 23:45:15 GMT</lastBuildDate> <generator>Silverchair</generator> <item> <title>Targeting lipid droplets in FUS-linked amyotrophic lateral sclerosis mitigates neuronal and astrocytic lipotoxicity</title> <link>https://academic.oup.com/brain/advance-article/doi/10.1093/brain/awaf328/8254939?rss=1</link> <pubDate>Tue, 16 Sep 2025 00:00:00 GMT</pubDate> <description><span class="paragraphSection"><div class="boxTitle">Abstract</div>Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder characterized by the progressive loss of motor neurons, muscle atrophy, and systemic energy imbalance. Increasing evidence suggests a metabolic shift in ALS from glucose metabolism toward fatty acid utilization; however, the downstream consequences of this reprogramming on disease progression and neuropathology remain poorly defined.We investigated neurometabolic changes in ALS using <span style="font-style:italic;">in vitro</span> and <span style="font-style:italic;">in vivo</span> models of familial ALS expressing the human <span style="font-style:italic;">fused in sarcoma</span> variant R521G (hFUS<sup>R521G</sup>), along with post-mortem spinal cord tissue from ALS-FUS cases. A combination of unbiased quantitative metabolomic profiling, immunolabeling, and biochemical and molecular approaches were employed.Mass spectrometry of cortical tissue from hFUS<sup>R521G</sup> mice and littermates revealed a significant increase in acylcarnitine moieties, key substrates used in mitochondrial β-oxidation and cellular energy production. Complementary cytohistological analyses in hFUS<sup>R521G</sup> mice demonstrated increased lipid droplets (LDs) and peroxidized lipids in both neurons and astrocytes, consistent with our post-mortem findings in spinal cords of individuals carrying FUS R495X or K510E mutations. Arimoclomol, previously shown to ameliorate behavioral phenotypes in this ALS mouse model, was found to enhance lipid metabolism and reduce lipotoxicity in hFUS<sup>R521G</sup> mice and in cultured neurons and astrocytes expressing FUS R521G. Mechanistically, arimoclomol enhanced LD-mitochondrial contacts and stimulated mitochondrial β-oxidation-dependent lipid catabolism under both basal and pro-inflammatory conditions. This effect was abrogated by etomoxir, an irreversible inhibitor of CPT1, the rate-limiting enzyme of the carnitine shuttle, highlighting a CPT1-dependent mechanism for lipid mobilization.Together, these findings reveal a previously unrecognized role for mitochondrial lipid metabolism in ALS pathogenesis and identify a therapeutic pathway for mitigating the cytotoxic consequences of lipid and acylcarnitine accumulation in FUS-associated ALS.</span></description> <prism:startingPage xmlns:prism="prism">awaf328</prism:startingPage> <prism:doi xmlns:prism="prism">10.1093/brain/awaf328</prism:doi> <guid>http://doi.org/10.1093/brain/awaf328</guid> </item> <item> <title>From intuitive guesswork to useful data for better clinical decisions</title> <link>https://academic.oup.com/brain/advance-article/doi/10.1093/brain/awaf343/8253752?rss=1</link> <pubDate>Sat, 13 Sep 2025 00:00:00 GMT</pubDate> <description><span class="paragraphSection"></span></description> <prism:startingPage xmlns:prism="prism">awaf343</prism:startingPage> <prism:doi xmlns:prism="prism">10.1093/brain/awaf343</prism:doi> <guid>http://doi.org/10.1093/brain/awaf343</guid> </item> </channel></rss>If you would like to create a banner that links to this page (i.e. this validation result), do the following:
Download the "valid RSS" banner.
Upload the image to your own server. (This step is important. Please do not link directly to the image on this server.)
Add this HTML to your page (change the image src attribute if necessary):
If you would like to create a text link instead, here is the URL you can use:
