Sorting of Von Willebrand factor to lysosome-related granules of haematopoietic cells
(2004) In Biochemical and Biophysical Research Communications 315(3). p.671-678- Abstract
- 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 (VWFm) were investigated after expression in the 32D cell line. Normal proteolytic processing was observed for both proteins, however the processing of VWFm was much slower and a large portion was unprocessed. Results from subcellular fractionation and immunoelectron microscopy confirmed that a part of VWF, but not VWFm, was targeted to lysosome-related granules. Partial constitutive secretion was also observed for all forms of VWF and VWFm. Inhibition of acidification by chloroquine blocked VWF processing but... (More)
- 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 (VWFm) were investigated after expression in the 32D cell line. Normal proteolytic processing was observed for both proteins, however the processing of VWFm was much slower and a large portion was unprocessed. Results from subcellular fractionation and immunoelectron microscopy confirmed that a part of VWF, but not VWFm, was targeted to lysosome-related granules. Partial constitutive secretion was also observed for all forms of VWF and VWFm. 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. (C) 2004 Elsevier Inc. All rights reserved. (Less)
Please use this url to cite or link to this publication:
https://lup.lub.lu.se/record/120465
- author
- Rosén, Hanna LU ; Calafat, Jero ; Holmberg, Lars LU and Olsson, Inge LU
- organization
- publishing date
- 2004
- type
- Contribution to journal
- publication status
- published
- subject
- in
- Biochemical and Biophysical Research Communications
- volume
- 315
- issue
- 3
- pages
- 671 - 678
- publisher
- Elsevier
- external identifiers
-
- wos:000189291900022
- scopus:1242340425
- pmid:14975753
- ISSN
- 1090-2104
- DOI
- 10.1016/j.bbrc.2004.01.106
- language
- English
- LU publication?
- yes
- id
- 53669d4f-9966-46a8-8d9d-bf895fe53675 (old id 120465)
- alternative location
- http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=14975753&dopt=Abstract
- date added to LUP
- 2016-04-01 15:17:54
- date last changed
- 2024-10-11 02:23:44
@article{53669d4f-9966-46a8-8d9d-bf895fe53675, abstract = {{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 (VWFm) were investigated after expression in the 32D cell line. Normal proteolytic processing was observed for both proteins, however the processing of VWFm was much slower and a large portion was unprocessed. Results from subcellular fractionation and immunoelectron microscopy confirmed that a part of VWF, but not VWFm, was targeted to lysosome-related granules. Partial constitutive secretion was also observed for all forms of VWF and VWFm. 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. (C) 2004 Elsevier Inc. All rights reserved.}}, author = {{Rosén, Hanna and Calafat, Jero and Holmberg, Lars and Olsson, Inge}}, issn = {{1090-2104}}, language = {{eng}}, number = {{3}}, pages = {{671--678}}, publisher = {{Elsevier}}, series = {{Biochemical and Biophysical Research Communications}}, title = {{Sorting of Von Willebrand factor to lysosome-related granules of haematopoietic cells}}, url = {{http://dx.doi.org/10.1016/j.bbrc.2004.01.106}}, doi = {{10.1016/j.bbrc.2004.01.106}}, volume = {{315}}, year = {{2004}}, }