Intracellular trafficking of Niemann–Pick C proteins 1 and 2: obligate components of subcellular lipid transport

Niemann–Pick C 1 (NPC1) is a large integral membrane glycoprotein that resides in late endosomes, whereas NPC2 is a small soluble protein found in the lumen of lysosomes. Mutations in either NPC1 or NPC2 result in aberrant lipid transport from endocytic compartments, which results in lysosomal stora...

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Bibliographic Details
Published inBiochimica et biophysica acta Vol. 1685; no. 1; pp. 22 - 27
Main Authors Liscum, Laura, Sturley, Stephen L.
Format Journal Article
LanguageEnglish
Published Netherlands Elsevier B.V 11.10.2004
Subjects
Animals
Carrier Proteins - metabolism
Endosomes - metabolism
Glycoproteins - metabolism
Humans
Lipid
Lipid Metabolism
Lysosomes - metabolism
Membrane Glycoproteins - metabolism
Models, Biological
Niemann-Pick Diseases - metabolism
Niemann–Pick C protein
Protein Transport
Trafficking
Trafficking
Lipid
Niemann–Pick C protein
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Summary:Niemann–Pick C 1 (NPC1) is a large integral membrane glycoprotein that resides in late endosomes, whereas NPC2 is a small soluble protein found in the lumen of lysosomes. Mutations in either NPC1 or NPC2 result in aberrant lipid transport from endocytic compartments, which results in lysosomal storage of a complex mixture of lipids, primarily cholesterol and glycosphingolipids. What are the biological functions of the NPC1 and NPC2 proteins? Here we review what is known about the intracellular itinerary of these two proteins as they facilitate lipid transport. We propose that the intracellular trafficking patterns of these proteins will provide clues about their function.
Bibliography:ObjectType-Article-2
SourceType-Scholarly Journals-1
ObjectType-Feature-3
content type line 23
ObjectType-Review-1
ISSN:1388-1981
0006-3002
1879-2618
DOI:10.1016/j.bbalip.2004.08.008