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rdf:type |
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lifeskim:mentions |
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pubmed:issue |
3
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pubmed:dateCreated |
2004-2-20
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pubmed:abstractText |
The aim of this work was to investigate sorting mechanisms of von Willebrand factor (VWF) when expressed in haematopoietic cells. The processing and sorting of both the wild-type VWF and a multimerization defective propeptide-mutant (VWF(m)) were investigated after expression in the 32D cell line. Normal proteolytic processing was observed for both proteins, however the processing of VWF(m) was much slower and a large portion was unprocessed. Results from subcellular fractionation and immunoelectron microscopy confirmed that a part of VWF, but not VWF(m), was targeted to lysosome-related granules. Partial constitutive secretion was also observed for all forms of VWF and VWF(m). Inhibition of acidification by chloroquine blocked VWF processing but allowed unprocessed pro-VWF targeting to dense organelles. In conclusion, our observations are consistent with VWF multimerization being of importance in cellular retention and targeting to lysosome-related organelles in haematopoietic cells, suggesting a role of protein aggregation for sorting in these cells.
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pubmed:language |
eng
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pubmed:journal |
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pubmed:citationSubset |
IM
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pubmed:chemical |
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pubmed:status |
MEDLINE
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pubmed:month |
Mar
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pubmed:issn |
0006-291X
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pubmed:author |
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pubmed:issnType |
Print
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pubmed:day |
12
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pubmed:volume |
315
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
671-8
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pubmed:dateRevised |
2006-11-15
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pubmed:meshHeading |
pubmed-meshheading:14975753-Animals,
pubmed-meshheading:14975753-Chloroquine,
pubmed-meshheading:14975753-Cytoplasmic Granules,
pubmed-meshheading:14975753-DNA, Complementary,
pubmed-meshheading:14975753-Endoplasmic Reticulum,
pubmed-meshheading:14975753-Frozen Sections,
pubmed-meshheading:14975753-Granulocyte Precursor Cells,
pubmed-meshheading:14975753-Lysosomes,
pubmed-meshheading:14975753-Mice,
pubmed-meshheading:14975753-Microscopy, Immunoelectron,
pubmed-meshheading:14975753-Mutation,
pubmed-meshheading:14975753-Precipitin Tests,
pubmed-meshheading:14975753-Protein Transport,
pubmed-meshheading:14975753-Recombinant Proteins,
pubmed-meshheading:14975753-Subcellular Fractions,
pubmed-meshheading:14975753-Sulfur Radioisotopes,
pubmed-meshheading:14975753-Transfection,
pubmed-meshheading:14975753-von Willebrand Factor
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pubmed:year |
2004
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pubmed:articleTitle |
Sorting of Von Willebrand factor to lysosome-related granules of haematopoietic cells.
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pubmed:affiliation |
Department of Hematology, C14, BMC, S-221 84 Lund, Sweden.
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pubmed:publicationType |
Journal Article,
Research Support, Non-U.S. Gov't
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