Results 201 to 210 of about 16,701 (225)

Conformational changes in the Niemann-Pick Type C1 protein NCR1 drive sterol translocation

2023
ABSTRACTThe membrane protein Niemann-Pick Type C1 protein (NPC1, named NCR1 in yeast) is central to sterol homeostasis in eukaryotes.Saccharomyces cerevisiaeNCR1 is localized to the vacuolar membrane, where it is suggested to carry sterols across the protective glycocalyx and deposit them into the vacuolar membrane. However, documentation of a vacuolar
Kelly M. Frain, Emil Dedic, Lynette Nel, Anastasiia Bohush, Esben Olesen, David L. Stokes, Bjørn Panyella Pedersen   +6 more
openaire   +1 more source

Structure of human Niemann‐Pick C1 (NPC1) protein and NPC1‐NPC2 complex

The FASEB Journal, 2017
Cholesterol, essential for all animal life, plays a vital role for maintenance of membrane strength and permeability, also serves as a precursor for the biosynthesis of lipoprotein, steroid hormones, bile acid and vitamin D. Our major cholesterol sources are endogenous biosynthesis and diet uptake.
openaire   +1 more source

Niemann Pick C1 mistargeting disrupts lysosomal cholesterol homeostasis contributing to neurodegeneration in a Batten disease model

Science Advances
Neurodegeneration is a devastating manifestation in most lysosomal storage disorders (LSDs). Loss-of-function mutations in CLN1, encoding palmitoyl-protein thioesterase-1 (PPT1), cause CLN1 disease, a devastating neurodegenerative LSD that has no ...
Abhilash P Appu, Maria B Bagh, Nisha Plavelil, Avisek Mondal, Tamal Sadhukhan, Satya P. Singh, Neil J Perkins, Aiyi Liu, Anil B Mukherjee   +8 more
semanticscholar   +1 more source

The Niemann-Pick C1 Protein Resides in a Vesicular Compartment Linked to Retrograde Transport of Multiple Lysosomal Cargo*

Journal of Biological Chemistry, 1999
E. Neufeld, M. Wastney, Shutish C. Patel, S. Suresh, A. Cooney, N. Dwyer, C. Roff, K. Ohno, Jill A. Morris, E. Carstea, J. Incardona, J. Strauss, M. Vanier, M. Patterson, R. Brady, P. Pentchev, E. Blanchette‐Mackie   +16 more
semanticscholar   +1 more source

Biliary lipid secretion in Niemann-Pick C1 protein-deficient mice

Gastroenterology, 2000
Silvana Zanlungo, Ludwig Amigo, Hegaly Mendoza, Francisco Morera, Juan Francisco Miquel, Attilio Rigotti, Flavio Nervi   +6 more
openaire   +1 more source

Niemann-Pick C1 Protein Facilitates the Efflux of the Anticancer Drug Daunorubicin from Cells According to a Novel Vesicle-Mediated Pathway

Journal of Pharmacology and Experimental Therapeutics, 2006
Y. Gong, M. Duvvuri, Michael B. Duncan, Jian Liu, J. Krise   +4 more
semanticscholar   +1 more source

Cytochrome b561, copper, &bgr;‐cleaved amyloid precursor protein and niemann‐pick C1 protein are involved in ascorbate‐induced release and membrane penetration of heparan sulfate from endosomal S‐nitrosylated glypican‐1

Experimental Cell Research, 2017
F. Cheng, L. Fransson, K. Mani
semanticscholar   +1 more source

Tonic prime-boost of STING signalling mediates Niemann–Pick disease type C

Nature, 2021
Xintao Tu, Kun Yang, Jianjun Wu
exaly  

NO-1886 Up-regulates Niemann–Pick C1 Protein (NPC1) Expression Through Liver X Receptor α Signaling Pathway in THP-1 Macrophage-Derived Foam Cells

Cardiovascular Drugs and Therapy, 2009
Xin Ma, Yan-wei Hu, Z. Mo, Xiao-xu Li, Xie-hong Liu, Ji Xiao, W. Yin, D. Liao, Chao-ke Tang   +8 more
semanticscholar   +1 more source

Intrathecal 2-hydroxypropyl-β-cyclodextrin decreases neurological disease progression in Niemann-Pick disease, type C1: a non-randomised, open-label, phase 1–2 trial

Lancet, The, 2017
Xuntian Jiang, Elizabeth M Berry-Kravis, Cristin D Davidson   +2 more
exaly