Mechanisms for cellular cholesterol transport: Defects and human disease
Abstract
This review summarizes the mechanisms of cellular cholesterol transport and monogenic human diseases caused by defects in intracellular cholesterol processing. In addition, selected mouse models of disturbed cholesterol trafficking are discussed. Current pharmacological strategies to prevent atherosclerosis are largely based on altering cellular cholesterol balance and are introduced in this context. Finally, because of the organizing potential of cholesterol in membranes, disturbances in cellular cholesterol transport have implications for a wide variety of human diseases, of which selected examples are given.
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MVA is the precursor of compounds other than cholesterol that are involved in transcription, protein N-glycosylation, protein prenylation, and mitochondrial electron transport.
“Importantly, MVA is the precursor of compounds other than cholesterol that are involved in diverse cellular processes such as transcription (isopentenyl tRNAs), protein N-glycosylation (dolichol phosphate), protein prenylation (farnesylation and geranylgeranylation), and mitochondrial electron transport (ubiquinone) (Figure 1) [17].”
From Loss of Mevalonate/Cholesterol Homeostasis in the Brain: A Focus on Autism Spectrum Disorder and Rett Syndrome (2019) · cites this paper as [17]
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References 291 · Cited by 229
- Authors
- Ikonen, E.
- Affiliations
- University of Helsinki (FI)
- Corresponding author
- Ikonen, Elina
- ORCID
- Ikonen, Elina
- Venue
- Physiological Reviews
- Publisher
- American Physiological Society
- Published
- 2006-10
- Volume
- 86
- Issue
- 4
- Pages
- 1237-1261
- ISSN
- 0031-9333 (ISSN-L)
- 1522-1210
- OpenAlex
- W2167584547
- Cited by
- 229
- References
- 291
- Citations by year
- 2026: 3 · 2025: 8 · 2024: 6 · 2023: 13 · 2022: 13 · 2021: 15 · 2020: 8 · 2019: 14 · 2018: 9 · 2017: 13 · 2016: 5 · 2015: 16 · 2014: 15 · 2013: 13 · 2012: 15
- FWCI
- 2.06
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Physiological Reviews (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Surgery › Cholesterol and Lipid Metabolism
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Lipid Membrane Structure and Behavior
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Cancer Research › Cancer, Lipids, and Metabolism
- Keywords
- cholesterol transport, atherosclerosis prevention, cholesterol metabolism, human disease
- MeSH terms
- Animals, Biological Transport, Cardiovascular Diseases, Cell Membrane, Cholesterol, Humans, Hypercholesterolemia, Intracellular Membranes, Steroids, Cytoplasmic Vesicles
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