| First Author | Sadhukhan T | Year | 2021 |
| Journal | Biochem Biophys Res Commun | Volume | 571 |
| Pages | 137-144 | PubMed ID | 34325129 |
| Mgi Jnum | J:321706 | Mgi Id | MGI:6765857 |
| Doi | 10.1016/j.bbrc.2021.07.057 | Citation | Sadhukhan T, et al. (2021) Ablation of microRNA-155 and neuroinflammation in a mouse model of CLN1-disease. Biochem Biophys Res Commun 571:137-144 |
| abstractText | Infantile neuronal ceroid lipofuscinosis (INCL), also known as CLN1-disease, is a devastating neurodegenerative lysosomal storage disorder (LSD), caused by inactivating mutations in the CLN1 gene. The Cln1(-/-) mice, which mimic INCL, manifest progressive neuroinflammation contributing to neurodegeneration. However, the underlying mechanism of neuroinflammation in INCL and in Cln1(-/-) mice has remained elusive. Previously, it has been reported that microRNA-155 (miR-155) regulates inflammation and miR profiling in Cln1(-/-) mouse brain showed that the level of miR-155 was upregulated. Thus, we sought to determine whether ablation of miR-155 in Cln1(-/-) mice may suppress neuroinflammation in these mice. Towards this goal, we generated Cln1(-/-)/miR-155(-/-) double-knockout mice and evaluated the inflammatory signatures in the brain. We found that the brains of double-KO mice manifest progressive neuroinflammatory changes virtually identical to those found in Cln1(-/-) mice. We conclude that ablation of miR-155 in Cln1(-/-) mice does not alter the neuroinflammatory trajectory in INCL mouse model. |
