Structural Insight into Eukaryotic Sterol Transport through Niemann-Pick Type C Proteins.
Mikael B. L. Winkler,Rune T. Kidmose,Maria Szomek,Katja Thaysen,Shaun Rawson,Stephen P. Muench,Daniel Wüstner,Bjørn Panyella Pedersen +7 more
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TLDR
A model for sterol integration is proposed that clarifies the role of NPC proteins in this essential eukaryotic pathway and that rationalizes mutations in patients with Niemann-Pick disease type C.About:
This article is published in Cell.The article was published on 2019-10-03 and is currently open access. It has received 97 citations till now. The article focuses on the topics: Sterol transport & Sterol homeostasis.read more
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NPC1-mTORC1 Signaling Couples Cholesterol Sensing to Organelle Homeostasis and Is a Targetable Pathway in Niemann-Pick Type C.
Oliver B. Davis,Hijai R. Shin,Chun-Yan Lim,Emma Y. Wu,Matthew Kukurugya,Claire F. Maher,Rushika M. Perera,M. Paulina Ordonez,Roberto Zoncu +8 more
TL;DR: Cholesterol-mTORC1 signaling controls organelle homeostasis and is a targetable pathway in Niemann-Pick type C and consistently ameliorates mitochondrial dysfunction in a neuronal model of NPC.
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Cholesterol Handling in Lysosomes and Beyond
TL;DR: This review summarizes the understanding of the major steps in cholesterol handling from the moment it enters the lysosome until it leaves this compartment.
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Structural Basis of Low-pH-Dependent Lysosomal Cholesterol Egress by NPC1 and NPC2.
TL;DR: In this article, structural characterizations reveal the molecular basis for low-pH-dependent cholesterol delivery from NPC2 to the transmembrane (TM) domain of NPC1.
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Cholesterol access in cellular membranes controls Hedgehog signaling.
TL;DR: The Hedgehog (Hh) receptor PTCH1 uses its transporter-like function to inhibit the GPCR SMO by limiting the pool of accessible membrane cholesterol, which acts as a ligand for SMO to activate downstream signaling.
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Mechanisms of nonvesicular lipid transport.
TL;DR: The role of non-vesicular lipid transport has been discussed in this article, where a review describes what we know about non-veto lipid transfer mechanisms while also identifying many remaining unknowns: How do LTPs facilitate lipid movement from and into membranes, do LTLTPs require accessory proteins for efficient transfer in vivo, and how is directionality of transport determined?
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