A new regulatory mechanism of STARD1 in Niemann-Pick disease type C (NPC), discovered
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Descrição
Researchers from the group Mitochondrial regulation of cell death and steatohepatitis have revealed a new role for acid ceramidase (ACDase) in experimental models and in patients with NPC through the regulation of STARD1 and its impact on mitochondrial function.
![A new regulatory mechanism of STARD1 in Niemann-Pick disease type C (NPC), discovered](https://onlinelibrary.wiley.com/cms/asset/2d85a149-cd32-46ea-8591-92a23bb597bc/jnc14895-fig-0002-m.jpg)
Niemann–Pick type C disease: cellular pathology and pharmacotherapy - Wheeler - 2020 - Journal of Neurochemistry - Wiley Online Library
![A new regulatory mechanism of STARD1 in Niemann-Pick disease type C (NPC), discovered](https://checkrare.com/wp-content/uploads/2020/01/CheckRare-Chart-1-Final-01-scaled.jpg)
Niemann–Pick Disease Type C
![A new regulatory mechanism of STARD1 in Niemann-Pick disease type C (NPC), discovered](https://upload.wikimedia.org/wikipedia/commons/thumb/9/99/Protein_NPC1_PDB_3GKH.png/800px-Protein_NPC1_PDB_3GKH.png)
NPC1 - Wikipedia
mRNA Treatment Rescues Niemann–Pick Disease Type C1 in Patient Fibroblasts
![A new regulatory mechanism of STARD1 in Niemann-Pick disease type C (NPC), discovered](https://www.embopress.org/cms/asset/d4816146-c0ff-4c4a-852f-c43145263d88/embr201948143-abs-0001-m.jpg)
NPC1 enables cholesterol mobilization during long‐term potentiation that can be restored in Niemann–Pick disease type C by CYP46A1 activation
![A new regulatory mechanism of STARD1 in Niemann-Pick disease type C (NPC), discovered](https://ars.els-cdn.com/content/image/1-s2.0-S2213231721002111-gr1.jpg)
Acid ceramidase improves mitochondrial function and oxidative stress in Niemann-Pick type C disease by repressing STARD1 expression and mitochondrial cholesterol accumulation - ScienceDirect
![A new regulatory mechanism of STARD1 in Niemann-Pick disease type C (NPC), discovered](https://ars.els-cdn.com/content/image/1-s2.0-S0003986116300315-fx1.jpg)
Mitochondrial dysfunction in fibroblasts derived from patients with Niemann-Pick type C disease - ScienceDirect
STARD1 and NPC1 expression as pathological markers associated with astrogliosis in post-mortem brains from patients with Alzheimer's disease and Down syndrome
![A new regulatory mechanism of STARD1 in Niemann-Pick disease type C (NPC), discovered](https://ars.els-cdn.com/content/image/1-s2.0-S2211124719305790-fx1.jpg)
Niemann-Pick Type C Disease Reveals a Link between Lysosomal Cholesterol and PtdIns(4,5)P2 That Regulates Neuronal Excitability - ScienceDirect
![A new regulatory mechanism of STARD1 in Niemann-Pick disease type C (NPC), discovered](https://www.irb.hr/var/ezflow_site/storage/images/media/multimedija-i-datoteke/zmm/lmnf/slide22/591002-1-eng-GB/Slide2_i1200.jpg)
Molecular mechanism(s) of neurodegeneration in Niemann-Pick type C disease - Ruđer Bošković Institute
![A new regulatory mechanism of STARD1 in Niemann-Pick disease type C (NPC), discovered](https://www.researchgate.net/publication/45660404/figure/fig1/AS:601660070055940@1520458347265/UPR-analysis-in-NPC-mice-cerebellum-a-NPC-and-wildtype-WT-mice-were-sacrificed-at-4-6.png)
UPR analysis in NPC mice cerebellum. a NPC and wildtype (WT) mice were
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