The DOI matches its Crossref record; 3 of the 3 references it cites were found in a registry or an index.
Structure of N-Terminal Domain of NPC1 Reveals Distinct Subdomains for Binding and Transfer of Cholesterol
Overview
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Integrity signals
Registry
Cites
Statistics
Verification
Claims the paper makes
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Claims other papers make about this one
Each is checked against this paper’s own text: does it say what the citing paper says it does?
NPC1 acts to enrich cholesterol in the lysosomal membrane.
“Molecular studies have established that NPC1 acts to enrich cholesterol in the lysosomal membrane (40, 41)”
From Aster-dependent nonvesicular transport facilitates dietary cholesterol uptake (2023) · cites this paper as [40]
The paper’s Abstract proposes that transfer from NPC2 to NPC1 allows cholesterol to insert into the lysosomal membrane. The Discussion says this membrane-insertion model remains unproven; it does not establish that NPC1 enriches cholesterol in that membrane. (LLM, conf=0.86)
Experimental — accuracy not yet measured
Self-check
Percentages, sums and reported differences re-computed from the paper’s own numbers.
The self-check needs the paper’s full text.
Citations
Cites
Coot : model-building tools for molecular graphics
Emsley, P., & Cowtan, K. (2004) · Acta Crystallographica Section D Biological Crystallography · doi:10.1107/s0907444904019158 · Cited by 30,571
A Receptor-Mediated Pathway for Cholesterol Homeostasis
Brown, M. S., & Goldstein, J. L. (1986) · Science · doi:10.1126/science.3513311 · Cited by 5,703
Self-citationCheruku, S. R., Xu, Z., Dutia, R., Lobel, P., & Storch, J. (2006) · Journal of Biological Chemistry · doi:10.1074/jbc.m602765200 · Cited by 204
Flagged first, then by citations
Research works printed but not linked: 39
- Andrews, 1965, The gel-filtration behaviour of proteins related to their molecular weights over a wide range, Biochem. J., 96, 595, 10.1042/bj0960595 · DOI
- Babalola, 2007, Development of an assay for the intermembrane transfer of cholesterol by Niemann-Pick C2 protein, Biol. Chem., 388, 617, 10.1515/BC.2007.063 · DOI
- Brown, 1997, The SREBP pathway: Regulation of cholesterol metabolism by proteolysis of a membrane-bound transcription factor, Cell, 89, 331, 10.1016/S0092-8674(00)80213-5 · DOI · Paper page
- Brunger, 1998, Crystallography & NMR system: A new software suite for macromolecular structure determination, Acta Crystallogr. D Biol. Crystallogr., D54, 905, 10.1107/S0907444998003254 · DOI
- Carstea, 1997, Niemann-Pick C1 disease gene: Homology to mediators of cholesterol homeostasis, Science, 277, 228, 10.1126/science.277.5323.228 · DOI · Paper page
- 1994, The CCP4 suite: Programs for protein crystallography, Acta Crystallogr. D Biol. Crystallogr., D50, 760
- Cowtan, 1994, Joint CCP4 and ESF-EACBM Newsletter on Protein Crystallography, 31, 34
- Dahl, 1992, Isolation and characterization of Chinese hamster ovary cells defective in the intracellular metabolism of low density lipoprotein-derived cholesterol, J. Biol. Chem., 267, 4889, 10.1016/S0021-9258(18)42914-6 · DOI
- Davies, 2000, J. Biol. Chem., 275, 24367, 10.1074/jbc.M002184200 · DOI
- DeBose-Boyd, 1999, Transport-dependent proteolysis of SREBP: Relocation of Site-1 protease from Golgi to ER obviates the need for SREBP transport to Golgi, Cell, 99, 703, 10.1016/S0092-8674(00)81668-2 · DOI · Paper page
- Demel, 1976, The function of sterols in membranes, Biochim. Biophys. Acta, 457, 109, 10.1016/0304-4157(76)90008-3 · DOI
- Friedland, 2003, Structure of a cholesterol-binding protein deficient in Niemann-Pick type C2 disease, Proc. Natl. Acad. Sci. USA, 100, 2512, 10.1073/pnas.0437840100 · DOI
- Goldstein, 1975, Role of lysosomal acid lipase in the metabolism of plasma low density lipoprotein: Observations in cultured fibroblasts from a patient with cholesteryl ester storage disease, J. Biol. Chem., 250, 8487, 10.1016/S0021-9258(19)40786-2 · DOI
- Goldstein, 2006, Protein sensors for membrane sterols, Cell, 124, 35, 10.1016/j.cell.2005.12.022 · DOI · Paper page
- Infante, 2008, Purified NPC1 protein: I. Binding of cholesterol and oxysterols to a 1278-amino acid membrane protein, J. Biol. Chem., 283, 1052, 10.1074/jbc.M707943200 · DOI
- Infante, 2008, Purified NPC1 protein: II. Localization of sterol binding to a 240-amino acid soluble luminal loop, J. Biol. Chem., 283, 1064, 10.1074/jbc.M707944200 · DOI
- Infante, 2008, NPC2 facilitates bidirectional transfer of cholesterol between NPC1 and lipid bilayers, a step in cholesterol egress from lysosomes, Proc. Natl. Acad. Sci. USA, 105, 15287, 10.1073/pnas.0807328105 · DOI
- Ko, 2003, The integrity of a cholesterol-binding pocket in Niemann-Pick C2 protein is necessary to control lysosome cholesterol levels, Proc. Natl. Acad. Sci. USA, 100, 2518, 10.1073/pnas.0530027100 · DOI
- Kolter, 2005, Principles of lysosomal membrane digestion: stimulation of sphingolipid degradation by sphingo-lipid activator proteins and anionic lysosomal lipids, Annu. Rev. Cell Dev. Biol., 21, 81, 10.1146/annurev.cellbio.21.122303.120013 · DOI · Paper page
- Liscum, 1987, Low density lipoprotein (LDL)-mediated suppression of cholesterol synthesis and LDL uptake is defective in Niemann-Pick type C fibroblasts, J. Biol. Chem., 262, 17002, 10.1016/S0021-9258(18)45483-X · DOI
- Naureckiene, 2000, Identification of HE1 as the second gene of Niemann-Pick C disease, Science, 290, 2298, 10.1126/science.290.5500.2298 · DOI
- Neiss, 1984, A coat of glycoconjugates on the inner surface of the lysosomal membrane in the rat kidney, Histochemistry, 80, 603, 10.1007/BF02400979 · DOI
- Ohgami, 2004, Binding between the Niemann-Pick C1 protein and a photoactivatable cholesterol analog requires a functional sterol-sensing domain, Proc. Natl. Acad. Sci. USA, 101, 12473, 10.1073/pnas.0405255101 · DOI
- Okamura, 1999, A porcine homolog of the major secretory protein of human epididymis, HE1, specifically binds cholesterol, Biochim. Biophys. Acta, 1438, 377, 10.1016/S1388-1981(99)00070-0 · DOI
- Otwinowski, 1997, Processing of x-ray diffraction data collected in oscillation mode, Methods Enzymol., 276, 307, 10.1016/S0076-6879(97)76066-X · DOI · Paper page
- Pentchev, 2004, Niemann-Pick C research from mouse to gene, Biochim. Biophys. Acta, 1685, 3, 10.1016/j.bbalip.2004.08.005 · DOI
- Radhakrishnan, 2004, Direct binding of cholesterol to the purified membrane region of SCAP: mechanism for a sterol-sensing domain, Mol. Cell, 15, 259, 10.1016/j.molcel.2004.06.019 · DOI · Paper page
- Roth, 2006, Clathrin-mediated endocytosis before fluorescent proteins, Nat. Rev. Mol. Cell Biol., 7, 63, 10.1038/nrm1783 · DOI
- Schneider, 2002, Substructure solution with SHELXD, Acta Crystallogr. D Biol. Crystallogr., D58, 1772, 10.1107/S0907444902011678 · DOI
- Schulze, 2009, Principles of lysosomal membrane degradation. Cellular topology and biochemistry of lysosomal lipid degradation, Biochim. Biophys. Acta, 1793, 674, 10.1016/j.bbamcr.2008.09.020 · DOI
- Simons, 2000, How cells handle cholesterol, Science, 290, 1721, 10.1126/science.290.5497.1721 · DOI
- Sleat, 2004, Genetic evidence for nonredundant functional cooperativity between NPC1 and NPC2 in lipid transport, Proc. Natl. Acad. Sci. USA, 101, 5886, 10.1073/pnas.0308456101 · DOI
- Subramanian, 2008, NPC1/NPC2 function as a tag team duo to mobilize cholesterol, Proc. Natl. Acad. Sci. USA, 105, 15223, 10.1073/pnas.0808256105 · DOI
- van Driel, 1987, First cysteine-rich repeat in ligand-binding domain of low density lipoprotein receptor binds Ca2+ and monoclonal antibodies, but not lipoproteins, J. Biol. Chem., 262, 17443, 10.1016/S0021-9258(18)45399-9 · DOI
- Wasilko, 2006, TIPS: Titerless infected-cells preservation and scale-up, Bioprocessing J., 5, 29, 10.12665/J53.WasilkoLee · DOI
- Watari, 1999, Niemann-Pick C1 protein: Obligatory roles for N-terminal domains and lysosomal targeting in cholesterol mobilization, Proc. Natl. Acad. Sci. USA, 96, 805, 10.1073/pnas.96.3.805 · DOI
- Winn, 2001, Use of TLS parameters to model anisotropic displacements in macromolecular refinement, Acta Crystallogr. D Biol. Crystallogr., D57, 122, 10.1107/S0907444900014736 · DOI
- Xu, 2007, Structural basis of sterol binding by NPC2, a lysosomal protein deficient in Niemann-Pick type C2 disease, J. Biol. Chem., 282, 23525, 10.1074/jbc.M703848200 · DOI
- Xu, 2008, Regulation of sterol transplant between membranes and NPC2, Biochemistry, 47, 11134, 10.1021/bi801328u · DOI
Cited by
Sorted by citations
Mechanisms and regulation of cholesterol homeostasis
Luo, J., Yang, H., & Song, B.-L. (2020) · Nature Reviews Molecular Cell Biology · doi:10.1038/s41580-019-0190-7 · Cited by 1,980
The lysosome: from waste bag to potential therapeutic target
Appelqvist, H., Wäster, P., Kågedal, K., & Öllinger, K. (2013) · Journal of Molecular Cell Biology · doi:10.1093/jmcb/mjt022 · Cited by 772
Lysosomal cholesterol activates mTORC1 via an SLC38A9–Niemann-Pick C1 signaling complex
Castellano, B. M., Thelen, A. M., Moldavski, O., Feltes, M., van der Welle, R. E. N., Mydock-McGrane, L., Jiang, X., van Eijkeren, R. J., Davis, O. B., Louie, S. M., et al. (2017) · Science · doi:10.1126/science.aag1417 · Cited by 562
Cholesterol metabolism in neurons and astrocytes
Pfrieger, F. W., & Ungerer, N. (2011) · Progress in Lipid Research · doi:10.1016/j.plipres.2011.06.002 · Cited by 500
ECOD: an evolutionary classification of protein domains
Cheng, H., Schaeffer, R. D., Liao, Y., Kinch, L. N., Pei, J., Shi, S., Kim, B.-H., & Grishin, N. V. (2014) · PLoS Computational Biology · doi:10.1371/journal.pcbi.1003926 · Cited by 426
“Together, the homologous cysteine-rich domains in Frizzled, NPC1, FRBP, and glypicans define a diverse superfamily of extracellular protein domains with an ancient eukaryotic origin and potential ligandbinding activities.”
BackgroundInfluentialAutophagy in lysosomal storage disorders
Lieberman, A. P., Puertollano, R., Raben, N., Slaugenhaupt, S., Walkley, S. U., & Ballabio, A. (2012) · Autophagy · doi:10.4161/auto.19469 · Cited by 399
Lu, F., Liang, Q., Abi-Mosleh, L., Das, A., De Brabander, J. K., Goldstein, J. L., & Brown, M. S. (2015) · eLife · doi:10.7554/elife.12177 · Cited by 333
Self-citationCholesterol in brain disease: Sometimes determinant and frequently implicated
Martín, M. G., Pfrieger, F., & Dotti, C. G. (2014) · The EMBO Reports · doi:10.15252/embr.201439225 · Cited by 304
Cholesterol and Alzheimer’s Disease; From Risk Genes to Pathological Effects
Feringa, F. M., & van der Kant, R. (2021) · Frontiers in Aging Neuroscience · doi:10.3389/fnagi.2021.690372 · Cited by 226
Disorders in the initial steps of steroid hormone synthesis
Miller, W. L. (2017) · The Journal of Steroid Biochemistry and Molecular Biology · doi:10.1016/j.jsbmb.2016.03.009 · Cited by 205
Trinh, M. N., Brown, M. S., Goldstein, J. L., Han, J., Vale, G., McDonald, J. G., Seemann, J., Mendell, J. T., & Lu, F. (2020) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.2010682117 · Cited by 129
Self-citationHead, S. A., Shi, W. Q., Yang, E. J., Nacev, B. A., Hong, S. Y., Pasunooti, K. K., Li, R.-J., Shim, J. S., & Liu, J. O. (2017) · ACS Chemical Biology · doi:10.1021/acschembio.6b00849 · Cited by 82
Lack of Niemann-Pick type C1 induces age-related degeneration in the mouse retina
Claudepierre, T., Paques, M., Simonutti, M., Buard, I., Sahel, J., Maue, R. A., Picaud, S., & Pfrieger, F. W. (2010) · Molecular and Cellular Neuroscience · doi:10.1016/j.mcn.2009.10.007 · Cited by 77
Reduction of lipid accumulation rescues Bietti's crystalline dystrophy phenotypes
Hata, M., Ikeda, H. O., Iwai, S., Iida, Y., Gotoh, N., Asaka, I., Ikeda, K., Isobe, Y., Hori, A., Nakagawa, S., et al. (2018) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1717338115 · Cited by 70
“NPC is a lipid storage disease caused by mutations in the NPC1 and NPC2 genes, which encode proteins involved in cholesterol transport between lysosomes and endoplasmic reticulum (43, 44).”
BackgroundAster-dependent nonvesicular transport facilitates dietary cholesterol uptake
Ferrari, A., Whang, E., Xiao, X., Kennelly, J. P., Romartinez-Alonso, B., Mack, J. J., Weston, T., Chen, K., Kim, Y., Tol, M. J., et al. (2023) · Science · doi:10.1126/science.adf0966 · Cited by 61
Role of Mitochondria in Steroidogenesis
Miller, W. L. (2011) · Endocrine Development · doi:10.1159/000321204 · Cited by 57
Midzak, A., & Papadopoulos, V. (2014) · Traffic · doi:10.1111/tra.12177 · Cited by 38
Allende, M. L., Zhu, H., Kono, M., Hoachlander-Hobby, L. E., Huso, V. L., & Proia, R. L. (2021) · Cellular Signalling · doi:10.1016/j.cellsig.2020.109879 · Cited by 37
Linden, A. K., Affaneh, A., Aylward, A., Wong, Y., Kessler, J. A., & Peng, C.-Y. (2025) · doi:10.64898/2025.12.14.694212 · Cited by 1
Metadata
- Authors
- Kwon, H. J., Abi-Mosleh, L., Wang, M. L., Deisenhofer, J., Goldstein, J. L., Brown, M. S., & Infante, R. E.
- Venue
- Cell
- Publisher
- Elsevier BV
- Published
- 2009-06
- Volume
- 137
- Issue
- 7
- Pages
- 1213-1224
- ISSN
- 0092-8674
- PMC
- PMC2739658
- Cited by
- 636
- References
- 3
- Language
- en
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
Are you an author, or do you see a problem with this page? Request a review