Results 61 to 70 of about 7,315 (135)

The ubiquitin‐proteasome system and autophagy as guardians of the cellular proteome

FEBS Letters, Volume 600, Issue 13, Page 1829-1841, July 2026.
This Perspective covers the three principles governing the crosstalk between the ubiquitin‐proteasome system and autophagy in cellular proteostasis: (1) a shared ubiquitin code routing substrates via shuttle factors or autophagy receptors; (2) spatial compartmentalization into phase‐separated degradation hubs and organelle‐specific modules (exemplified
Ivan Dikic
wiley   +1 more source

Modeling Niemann-Pick disease type C1 in zebrafish: a robust platform for in vivo screening of candidate therapeutic compounds

Disease Models & Mechanisms, 2018
Niemann-Pick disease type C1 (NPC1) is a rare autosomal recessive lysosomal storage disease primarily caused by mutations in NPC1. NPC1 is characterized by abnormal accumulation of unesterified cholesterol and glycolipids in late endosomes and lysosomes.
Wei-Chia Tseng, Hannah E. Loeb, Wuhong Pei, Chon-Hwa Tsai-Morris, Lisha Xu, Celine V. Cluzeau, Christopher A. Wassif, Benjamin Feldman, Shawn M. Burgess, William J. Pavan, Forbes D. Porter   +10 more
doaj   +1 more source

Altered vitamin E status in Niemann-Pick type C disease

Journal of Lipid Research, 2011
Vitamin E (α-tocopherol) is the major lipid-soluble antioxidant in many species. Niemann-Pick type C (NPC) disease is a lysosomal storage disorder caused by mutations in the NPC1 or NPC2 gene, which regulates lipid transport through the endocytic pathway.
L. Ulatowski, R. Parker, C. Davidson, N. Yanjanin, T.J. Kelley, D. Corey, J. Atkinson, F. Porter, H. Arai, S.U. Walkley, D. Manor   +10 more
doaj   +1 more source

The adenosine A2A receptor agonist T1–11 ameliorates neurovisceral symptoms and extends the lifespan of a mouse model of Niemann-Pick type C disease

Neurobiology of Disease, 2018
Niemann-Pick C is a fatal neurovisceral disorder caused, in 95% of cases, by mutation of NPC1 gene. Therapeutic options are extremely limited and new “druggable” targets are highly warranted.
Antonella Ferrante, Antonella Pezzola, Andrea Matteucci, Antonella Di Biase, Lucilla Attorri, Monica Armida, Alberto Martire, Yijuang Chern, Patrizia Popoli   +8 more
doaj   +1 more source

Menstrual blood-derived endometrial stem cells ameliorate neuroinflammation and apoptosis through JAK2/STAT3 signaling pathway in NPC1 mutant cell and mice

Stem Cell Research & Therapy
Background Niemann-Pick disease type C1 (NPC1) is caused by NPC1 gene mutations, resulting in Purkinje cell degeneration and death, glial cell activation, and progressive neurodegeneration.
Lihong Guan, Keli Xu, Minlin Yang, Yangyang Li, Xiaoying Li, Han Li, Ciqing Yang, Liang Qiao, Yanli Liu, Zisen Jia, Jinghui Wei, Fen Yang, Juntang Lin   +12 more
doaj   +1 more source

NPC1 as a novel therapeutic target for induction of pyroptosis in cancers

Biomarker Research
Background Cholesterol metabolism reprogramming is a hallmark of cancer cells that exhibit cholesterol addiction by absorbing low-density lipoprotein (LDL) to generate cholesterol for growth. Yet the underlying mechanisms remain unclear. Methods We began
Chuanchao Zhang, Qiang Wang, Pan Su, Jianfei Qian, Qi Guo, Wei Wu, Rui Duan, Yufei Zhang, Youli Zu, Ling Zhong, Liuling Xiao, Qing Yi   +11 more
doaj   +1 more source

ORMDL mislocalization by impaired autophagy in Niemann-Pick type C disease leads to increased de novo sphingolipid biosynthesis

Journal of Lipid Research
Niemann-Pick type C1 (NPC1) disease is a rare neurodegenerative cholesterol and sphingolipid storage disorder primarily due to mutations in the cholesterol-trafficking protein NPC1.
Ryan D.R. Brown, Usha Mahawar, Binks W. Wattenberg, Sarah Spiegel   +3 more
doaj   +1 more source

NPC1 controls TGFBR1 stability in a cholesterol transport-independent manner and promotes hepatocellular carcinoma progression

Nature Communications
Niemann-Pick disease type C protein 1 (NPC1), classically associated with cholesterol transport and viral entry, has an emerging role in cancer biology.
Shuangyan Li, Lishan Yan, Chaoying Li, Lijuan Lou, Fengjiao Cui, Xiao Yang, Fuchu He, Ying Jiang   +7 more
doaj   +1 more source

NPC1 promotes the progression of hepatocellular carcinoma by mediating the accumulation of neutrophils into the tumor microenvironment

FEBS Open Bio
Hepatocellular carcinoma remains a significant threat to human health. Recent studies have found that the intake of cellular cholesterol contributes to the development and progression of hepatocellular carcinoma, although the exact mechanisms remain ...
Songhai Yang, Jiangming Chen, Kun Xie, Fubao Liu   +3 more
doaj   +1 more source

Lack of cathepsin B increases severity in Niemann-Pick disease, type C1 mice. [PDF]

Neurosci Lett
Luke R, Cawley NX, Porter FD.
europepmc   +1 more source