NPC1-mTORC1 Signaling Couples Cholesterol Sensing to Organelle Homeostasis and Is a Targetable Pathway in Niemann-Pick Type C
Lysosomes promote cellular homeostasis through macromolecular hydrolysis within their lumen and metabolic signaling by the mTORC1 kinase on their limiting membranes. Both hydrolytic and signaling functions require precise regulation of lysosomal cholesterol content. In Niemann-Pick type C (NPC), los...
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| Published in | Developmental cell Vol. 56; no. 3; pp. 260 - 276.e7 |
|---|---|
| Main Authors | , , , , , , , , |
| Format | Journal Article |
| Language | English |
| Published |
United States
Elsevier Inc
08.02.2021
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| Subjects | |
| Online Access | Get full text |
| ISSN | 1534-5807 1878-1551 1878-1551 |
| DOI | 10.1016/j.devcel.2020.11.016 |
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| Abstract | Lysosomes promote cellular homeostasis through macromolecular hydrolysis within their lumen and metabolic signaling by the mTORC1 kinase on their limiting membranes. Both hydrolytic and signaling functions require precise regulation of lysosomal cholesterol content. In Niemann-Pick type C (NPC), loss of the cholesterol exporter, NPC1, causes cholesterol accumulation within lysosomes, leading to mTORC1 hyperactivation, disrupted mitochondrial function, and neurodegeneration. The compositional and functional alterations in NPC lysosomes and nature of aberrant cholesterol-mTORC1 signaling contribution to organelle pathogenesis are not understood. Through proteomic profiling of NPC lysosomes, we find pronounced proteolytic impairment compounded with hydrolase depletion, enhanced membrane damage, and defective mitophagy. Genetic and pharmacologic mTORC1 inhibition restores lysosomal proteolysis without correcting cholesterol storage, implicating aberrant mTORC1 as a pathogenic driver downstream of cholesterol accumulation. Consistently, mTORC1 inhibition ameliorates mitochondrial dysfunction in a neuronal model of NPC. Thus, cholesterol-mTORC1 signaling controls organelle homeostasis and is a targetable pathway in NPC.
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•Proteomic profiling of NPC lysosomes reveals both proteolytic and structural defects•Loss of cholesterol transport activity by NPC1 causes aberrant mTORC1 signaling•mTORC1 inhibition restores lysosomal and mitochondrial function in NPC cells
Niemann-Pick type C is a devastating neurodegenerative disease caused by cholesterol buildup in lysosomes. Through organelle proteomics, Davis et al. identify degradative and structural defects of NPC lysosomes. Aberrant mTORC1 signaling drives lysosomal dysfunction downstream of cholesterol accumulation, and chemical and genetic mTORC1 inhibition restores organelle homeostasis in NPC cells. |
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| AbstractList | Lysosomes promote cellular homeostasis through macromolecular hydrolysis within their lumen and metabolic signaling by the mTORC1 kinase on their limiting membranes. Both hydrolytic and signaling functions require precise regulation of lysosomal cholesterol content. In Niemann-Pick type C (NPC), loss of the cholesterol exporter, NPC1, causes cholesterol accumulation within lysosomes, leading to mTORC1 hyperactivation, disrupted mitochondrial function, and neurodegeneration. The compositional and functional alterations in NPC lysosomes and nature of aberrant cholesterol-mTORC1 signaling contribution to organelle pathogenesis are not understood. Through proteomic profiling of NPC lysosomes, we find pronounced proteolytic impairment compounded with hydrolase depletion, enhanced membrane damage, and defective mitophagy. Genetic and pharmacologic mTORC1 inhibition restores lysosomal proteolysis without correcting cholesterol storage, implicating aberrant mTORC1 as a pathogenic driver downstream of cholesterol accumulation. Consistently, mTORC1 inhibition ameliorates mitochondrial dysfunction in a neuronal model of NPC. Thus, cholesterol-mTORC1 signaling controls organelle homeostasis and is a targetable pathway in NPC. Lysosomes promote cellular homeostasis through macromolecular hydrolysis within their lumen and metabolic signaling by the mTORC1 kinase on their limiting membranes. Both hydrolytic and signaling functions require precise regulation of lysosomal cholesterol content. In Niemann-Pick type C (NPC), loss of the cholesterol exporter, NPC1, causes cholesterol accumulation within lysosomes, leading to mTORC1 hyperactivation, disrupted mitochondrial function and neurodegeneration. The compositional and functional alterations in NPC lysosomes, and how aberrant cholesterol-mTORC1 signaling contributes to organelle pathogenesis are not understood. Through proteomic profiling of NPC lysosomes, we find pronounced proteolytic impairment compounded with hydrolase depletion, enhanced membrane damage and defective mitophagy. Genetic and pharmacologic mTORC1 inhibition restores lysosomal proteolysis without correcting cholesterol storage, implicating aberrant mTORC1 as a pathogenic driver downstream of cholesterol accumulation. Consistently, mTORC1 inhibition ameliorates mitochondrial dysfunction in a neuronal model of NPC. Thus, cholesterol-mTORC1 signaling controls organelle homeostasis and is a targetable pathway in NPC. Niemann-Pick type C is a devastating neurodegenerative disease caused by cholesterol buildup in lysosomes. Through organelle proteomics, Davis et al identify degradative and structural defects of NPC lysosomes. Aberrant mTORC1 signaling drives lysosomal dysfunction downstream of cholesterol accumulation, and chemical and genetic mTORC1 inhibition restores organelle homeostasis in NPC cells. Lysosomes promote cellular homeostasis through macromolecular hydrolysis within their lumen and metabolic signaling by the mTORC1 kinase on their limiting membranes. Both hydrolytic and signaling functions require precise regulation of lysosomal cholesterol content. In Niemann-Pick type C (NPC), loss of the cholesterol exporter, NPC1, causes cholesterol accumulation within lysosomes, leading to mTORC1 hyperactivation, disrupted mitochondrial function, and neurodegeneration. The compositional and functional alterations in NPC lysosomes and nature of aberrant cholesterol-mTORC1 signaling contribution to organelle pathogenesis are not understood. Through proteomic profiling of NPC lysosomes, we find pronounced proteolytic impairment compounded with hydrolase depletion, enhanced membrane damage, and defective mitophagy. Genetic and pharmacologic mTORC1 inhibition restores lysosomal proteolysis without correcting cholesterol storage, implicating aberrant mTORC1 as a pathogenic driver downstream of cholesterol accumulation. Consistently, mTORC1 inhibition ameliorates mitochondrial dysfunction in a neuronal model of NPC. Thus, cholesterol-mTORC1 signaling controls organelle homeostasis and is a targetable pathway in NPC. [Display omitted] •Proteomic profiling of NPC lysosomes reveals both proteolytic and structural defects•Loss of cholesterol transport activity by NPC1 causes aberrant mTORC1 signaling•mTORC1 inhibition restores lysosomal and mitochondrial function in NPC cells Niemann-Pick type C is a devastating neurodegenerative disease caused by cholesterol buildup in lysosomes. Through organelle proteomics, Davis et al. identify degradative and structural defects of NPC lysosomes. Aberrant mTORC1 signaling drives lysosomal dysfunction downstream of cholesterol accumulation, and chemical and genetic mTORC1 inhibition restores organelle homeostasis in NPC cells. Lysosomes promote cellular homeostasis through macromolecular hydrolysis within their lumen and metabolic signaling by the mTORC1 kinase on their limiting membranes. Both hydrolytic and signaling functions require precise regulation of lysosomal cholesterol content. In Niemann-Pick type C (NPC), loss of the cholesterol exporter, NPC1, causes cholesterol accumulation within lysosomes, leading to mTORC1 hyperactivation, disrupted mitochondrial function, and neurodegeneration. The compositional and functional alterations in NPC lysosomes and nature of aberrant cholesterol-mTORC1 signaling contribution to organelle pathogenesis are not understood. Through proteomic profiling of NPC lysosomes, we find pronounced proteolytic impairment compounded with hydrolase depletion, enhanced membrane damage, and defective mitophagy. Genetic and pharmacologic mTORC1 inhibition restores lysosomal proteolysis without correcting cholesterol storage, implicating aberrant mTORC1 as a pathogenic driver downstream of cholesterol accumulation. Consistently, mTORC1 inhibition ameliorates mitochondrial dysfunction in a neuronal model of NPC. Thus, cholesterol-mTORC1 signaling controls organelle homeostasis and is a targetable pathway in NPC.Lysosomes promote cellular homeostasis through macromolecular hydrolysis within their lumen and metabolic signaling by the mTORC1 kinase on their limiting membranes. Both hydrolytic and signaling functions require precise regulation of lysosomal cholesterol content. In Niemann-Pick type C (NPC), loss of the cholesterol exporter, NPC1, causes cholesterol accumulation within lysosomes, leading to mTORC1 hyperactivation, disrupted mitochondrial function, and neurodegeneration. The compositional and functional alterations in NPC lysosomes and nature of aberrant cholesterol-mTORC1 signaling contribution to organelle pathogenesis are not understood. Through proteomic profiling of NPC lysosomes, we find pronounced proteolytic impairment compounded with hydrolase depletion, enhanced membrane damage, and defective mitophagy. Genetic and pharmacologic mTORC1 inhibition restores lysosomal proteolysis without correcting cholesterol storage, implicating aberrant mTORC1 as a pathogenic driver downstream of cholesterol accumulation. Consistently, mTORC1 inhibition ameliorates mitochondrial dysfunction in a neuronal model of NPC. Thus, cholesterol-mTORC1 signaling controls organelle homeostasis and is a targetable pathway in NPC. |
| Author | Davis, Oliver B. Maher, Claire F. Shin, Hijai R. Perera, Rushika M. Kukurugya, Matthew Lim, Chun-Yan Wu, Emma Y. Zoncu, Roberto Ordonez, M. Paulina |
| AuthorAffiliation | 2 The Paul F. Glenn Center for Aging Research at the University of California, Berkeley, Berkeley, CA 94720, USA 3 Department of Anatomy and Helen Diller Family Comprehensive Cancer center, University of California, San Francisco, San Francisco, CA 94143, USA 4 Sanford Consortium for Regenerative Medicine, University of California, San Diego, La Jolla, CA 92037, USA 6 Lead Contact 1 Department of Molecular and Cell Biology, University of California, Berkeley, Berkeley, CA 94720, USA 5 Department of Pediatric Gastroenterology, Hepatology, and Nutrition, University of California, San Diego, La Jolla, CA 92037, USA |
| AuthorAffiliation_xml | – name: 1 Department of Molecular and Cell Biology, University of California, Berkeley, Berkeley, CA 94720, USA – name: 3 Department of Anatomy and Helen Diller Family Comprehensive Cancer center, University of California, San Francisco, San Francisco, CA 94143, USA – name: 6 Lead Contact – name: 2 The Paul F. Glenn Center for Aging Research at the University of California, Berkeley, Berkeley, CA 94720, USA – name: 4 Sanford Consortium for Regenerative Medicine, University of California, San Diego, La Jolla, CA 92037, USA – name: 5 Department of Pediatric Gastroenterology, Hepatology, and Nutrition, University of California, San Diego, La Jolla, CA 92037, USA |
| Author_xml | – sequence: 1 givenname: Oliver B. surname: Davis fullname: Davis, Oliver B. organization: Department of Molecular and Cell Biology, University of California, Berkeley, Berkeley, CA 94720, USA – sequence: 2 givenname: Hijai R. surname: Shin fullname: Shin, Hijai R. organization: Department of Molecular and Cell Biology, University of California, Berkeley, Berkeley, CA 94720, USA – sequence: 3 givenname: Chun-Yan surname: Lim fullname: Lim, Chun-Yan organization: Department of Molecular and Cell Biology, University of California, Berkeley, Berkeley, CA 94720, USA – sequence: 4 givenname: Emma Y. surname: Wu fullname: Wu, Emma Y. organization: Sanford Consortium for Regenerative Medicine, University of California, San Diego, La Jolla, CA 92037, USA – sequence: 5 givenname: Matthew surname: Kukurugya fullname: Kukurugya, Matthew organization: Department of Molecular and Cell Biology, University of California, Berkeley, Berkeley, CA 94720, USA – sequence: 6 givenname: Claire F. surname: Maher fullname: Maher, Claire F. organization: Department of Molecular and Cell Biology, University of California, Berkeley, Berkeley, CA 94720, USA – sequence: 7 givenname: Rushika M. surname: Perera fullname: Perera, Rushika M. organization: Department of Anatomy and Helen Diller Family Comprehensive Cancer Center, University of California, San Francisco, San Francisco, CA 94143, USA – sequence: 8 givenname: M. Paulina surname: Ordonez fullname: Ordonez, M. Paulina email: pordonez@health.ucsd.edu organization: Sanford Consortium for Regenerative Medicine, University of California, San Diego, La Jolla, CA 92037, USA – sequence: 9 givenname: Roberto surname: Zoncu fullname: Zoncu, Roberto email: rzoncu@berkeley.edu organization: Department of Molecular and Cell Biology, University of California, Berkeley, Berkeley, CA 94720, USA |
| BackLink | https://www.ncbi.nlm.nih.gov/pubmed/33308480$$D View this record in MEDLINE/PubMed |
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| Keywords | autophagy NPC1 ESCRT proteomics mitochondria cholesterol mTORC1 lysosome proteolysis |
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| Notes | ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23 O.B.D, M.P.O., and R.Z. conceived of and designed experiments. O.B.D, H.R.S., C.Y.L., E.Y.W., M.K., C.F.M. and M.P.O. generated key reagents and performed the experiments. O.B.D, H.R.S., R.M.P., M.P.O. and R.Z. analyzed data and interpreted results. O.B.D., M.P.O and R.Z. wrote the manuscript. Author Contributions All authors read and edited the manuscript. |
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| PublicationYear | 2021 |
| Publisher | Elsevier Inc |
| Publisher_xml | – name: Elsevier Inc |
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| SubjectTerms | Adult Animals autophagy Cells, Cultured cholesterol Cholesterol - metabolism ESCRT HEK293 Cells Homeostasis Humans Induced Pluripotent Stem Cells - metabolism Intracellular Membranes - metabolism Intracellular Signaling Peptides and Proteins - metabolism lysosome Lysosomes - metabolism Mechanistic Target of Rapamycin Complex 1 - metabolism Mice mitochondria Mitochondria - metabolism Models, Biological mTORC1 Neurons - metabolism Niemann-Pick C1 Protein Niemann-Pick Disease, Type C - metabolism NPC1 Organelles - metabolism Proteolysis proteomics Signal Transduction |
| Title | NPC1-mTORC1 Signaling Couples Cholesterol Sensing to Organelle Homeostasis and Is a Targetable Pathway in Niemann-Pick Type C |
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