Cited by 221Lysosomal Storage Disorders ResearchAutophagy in Disease and TherapyGlycogen Storage Diseases and Myoclonus

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Cholesterol Accumulation Is Associated with Lysosomal Dysfunction and Autophagic Stress in Npc1−/− Mouse Brain

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Liao, Guanghong, Yao, Yueqin, Liu, Jihua, Yu, Zhang, Cheung, Simon, Xie, Ang, Liang, Xiaoli, Bi, Xiaoning·2007·The American Journal of Pathology

DOI 10.2353/ajpath.2007.070052PMC PMC1959498

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Abstract

Niemann-Pick type C (NPC) disease is an autosomal recessive disorder caused by mutations of NPC1 and NPC2 genes. Progressive neurodegeneration that accompanies NPC is fatal, but the underlying mechanisms are still poorly understood. In the present study, we characterized the association of autophagic-lysosomal dysfunction with cholesterol accumulation in Npc1(-/-) mice during postnatal development. Brain levels of lysosomal cathepsin D were significantly higher in mutant than in wild-type mice. Increases in cathepsin D occurred first in neurons and later in astrocytes and microglia and were both spatially and temporally associated with intracellular cholesterol accumulation and neurodegeneration. Furthermore, levels of ubiquitinated proteins were higher in endosomal/lysosomal fractions of brains from Npc1(-/-) mice than from wild-type mice. Immunoblotting results showed that levels of LC3-II were significantly higher in brains of mutant than wild-type mice. Combined LC3 immunofluorescence and filipin staining showed that LC3 accumulated within filipin-labeled cholesterol clusters inside Purkinje cells. Electron microscopic examination revealed the existence of autophagic vacuole-like structures and multivesicles in brains from Npc1(-/-) mice. These results provide strong evidence that cholesterol accumulation-induced changes in autophagy-lysosome function are closely associated with neurodegeneration in NPC.

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Authors: Liao, G., Yao, Y., Liu, J., Yu, Z., Cheung, S., Xie, A., Liang, X., & Bi, X.
Affiliations: Western University of Health Sciences (US)
University of California, Irvine (US)
Academy of Military Medical Sciences (CN)
Corresponding author: Bi, Xiaoning
ORCID: Liao, Guanghong
Liu, Jihua
Liang, Xiaoli
Venue: The American Journal of Pathology
Publisher: Elsevier BV
Published: 2007-09
Volume: 171
Issue: 3
Pages: 962-975
ISSN: 0002-9440 (ISSN-L)
DOI: 10.2353/ajpath.2007.070052
PMC: PMC1959498
OpenAlex: W2054595666
Cited by: 220
References: 73
Citations by year: 2026: 2 · 2025: 6 · 2024: 14 · 2023: 10 · 2022: 9 · 2021: 12 · 2020: 12 · 2019: 12 · 2018: 10 · 2017: 9 · 2016: 13 · 2015: 10 · 2014: 10 · 2013: 16 · 2012: 10
FWCI: 12.08
Type: journal article
Language: en
Open access: bronze
doi.org
Licence: www.elsevier.com/tdm/userlicense/1.0/ (tdm)
Locations: American Journal Of Pathology (publishedVersion)
PubMed Central (publishedVersion)
PubMed (publishedVersion)
Funders: National Institutes of Health
National Institute of Neurological Disorders and Stroke (R01NS048423, NS048423)
Institute of Genetics
Topics: Health Sciences › Medicine › Genetics (clinical) › Lysosomal Storage Disorders Research
Life Sciences › Biochemistry, Genetics and Molecular Biology › Cell Biology › Autophagy in Disease and Therapy
Life Sciences › Biochemistry, Genetics and Molecular Biology › Biochemistry › Glycogen Storage Diseases and Myoclonus
Keywords: Niemann-Pick disease type C, Npc1 knockout mice, intracellular cholesterol accumulation, cathepsin D, neurodegeneration, ubiquitinated proteins, LC3, autophagosomes, Purkinje cells, astrocytes, microglia, postnatal brain development
MeSH terms: Niemann-Pick C1 Protein, Animals, Autophagy, Brain, Cathepsin B, Cathepsin D, Child, Cholesterol, Filipin, Humans, Lysosomes, Mice, Inbred BALB C, Microtubule-Associated Proteins, Niemann-Pick Diseases, Proteins, Purkinje Cells, Mice, Knockout, Intracellular Signaling Peptides and Proteins, Mice

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Cholesterol Accumulation Is Associated with Lysosomal Dysfunction and Autophagic Stress in Npc1−/− Mouse Brain

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