Binding between the Niemann-Pick C1 protein and a photoactivatable cholesterol analog requires a functional sterol-sensing domain.

No abstract is available for this paper.

• Publication year

  2004

• Venue

  Proceedings of the National Academy of Sciences of the United States of America

• Publication date

  2004-08-24

• Fields of study

  Biology, Medicine, Chemistry

• Identifiers
  DOI 10.1073/PNAS.0405255101PMID 15314240PMCID PMC514655
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

• The Metabolic and Molecular Bases of Inherited Disease (Scriver, C. R., Beaudet, A. L., Sly, W. S., Valle, D., Childs, B., Kinzler, K. W., and Vogelstein, B., eds., 8th ed., McGraw-Hill, New-York, 2001, 7012 p., $550.00)

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• Circulation of Cholesterol between Lysosomes and the Plasma Membrane*

  1998cited by this paper
• Niemann-Pick C1 disease gene: homology to mediators of cholesterol homeostasis.

  1997cited by this paper
• Sterol resistance in CHO cells traced to point mutation in SREBP cleavage-activating protein.

  1996cited by this paper
• Use of photocrosslinkers in cell biology.

  1996cited by this paper
• Isolation and characterization of Chinese hamster ovary cells defective in the intracellular metabolism of low density lipoprotein-derived cholesterol.

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  1991cited by this paper
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  1990cited by this paper
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  1990cited by this paper
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  1989cited by this paper
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