The DOI matches its Crossref record, with a small difference in title or year; 48 of the 48 references it cites were found in a registry or an index.
Chondroitin Sulfate Perlecan Enhances Collagen Fibril Formation
Open accessAbstract
Inactivation of the perlecan gene leads to perinatal lethal chondrodysplasia. The similarity to the phenotypes of the Col2A1 knock-out and the disproportionate micromelia mutation suggests perlecan involvement in cartilage collagen matrix assembly. We now present a mechanism for the defect in collagen type II fibril assembly by perlecan-null chondrocytes. Cartilage perlecan is a heparin sulfate or a mixed heparan sulfate/chondroitin sulfate proteoglycan. The latter form binds collagen and accelerates fibril formation in vitro, with more defined fibril morphology and increased fibril diameters produced in the presence of perlecan. Interestingly, the enhancement of collagen fibril formation is independent on the core protein and is mimicked by chondroitin sulfate E but neither by chondroitin sulfate D nor dextran sulfate. Furthermore, perlecan chondroitin sulfate contains the 4,6-disulfated disaccharides typical for chondroitin sulfate E. Indeed, purified glycosaminoglycans from perlecan-enriched fractions of cartilage extracts contain elevated levels of 4,6-disulfated chondroitin sulfate disaccharides and enhance collagen fibril formation. The effect on collagen assembly is proportional to the content of the 4,6-disulfated disaccharide in the different cartilage extracts, with growth plate cartilage glycosaminoglycan being the most efficient enhancer. These findings demonstrate a role for perlecan chondroitin sulfate side chains in cartilage extracellular matrix assembly and provide an explanation for the perlecan-null chondrodysplasia.
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“HSPG2 encodes perlecan, a PG present in the basement membranes, which binds to and cross-links many ECM components and cell-surface proteins, such as COLLI, COLLIV, laminin, nidogen, and FN [31].”
Citations
Cites
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A Direct Spectrophotometric Microassay for Sulfated Glycosaminoglycans in Cartilage Cultures
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Self-citationPerlecan Maintains the Integrity of Cartilage and Some Basement Membranes
Costell, M., Gustafsson, E., Aszódi, A., Mörgelin, M., Bloch, W., Hunziker, E. B., Addicks, K., Timpl, R., & Fässler, R. (1999) · The Journal of Cell Biology · doi:10.1083/jcb.147.5.1109 · Cited by 672
Self-citationAviezer, D., Hecht, D., Safran, M., Eisinger, M., David, G., & Yayon, A. (1994) · Cell · doi:10.1016/0092-8674(94)90031-0 · Cited by 546
Perlecan is essential for cartilage and cephalic development
Arikawa‐Hirasawa, E., Watanabe, H., Takami, H., Hassell, J. R., & Yamada, Y. (1999) · Nature Genetics · doi:10.1038/15537 · Cited by 542
Phenotypic stability of bovine articular chondrocytes after long-term culture in alginate beads
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Amodei, L., Ruggieri, A. G., Potenza, F., Viele, M., Dufrusine, B., Franciotti, R., Pietrangelo, L., Ardini, M., Stuppia, L., Federici, L., et al. (2024) · Figshare · doi:10.6084/m9.figshare.26942763
Amodei, L., Ruggieri, A. G., Potenza, F., Viele, M., Dufrusine, B., Franciotti, R., Pietrangelo, L., Ardini, M., Stuppia, L., Federici, L., et al. (2024) · Figshare · doi:10.6084/m9.figshare.26825483
Amodei, L., Ruggieri, A. G., Potenza, F., Viele, M., Dufrusine, B., Franciotti, R., Pietrangelo, L., Ardini, M., Stuppia, L., Federici, L., et al. (2024) · Figshare · doi:10.6084/m9.figshare.26825474
Norris, G. E., & Parry, D. A. D. (2026) · Sub-cellular biochemistry/Subcellular biochemistry · doi:10.1007/978-3-032-05273-5_9
CRISPR screen uncovers SLC26A2 as a modulator of tungsten toxicity in endochondral ossification
Bakadlag, R., Li, S., Guilbert, C., Huard, C., Bertomeu, T., Coulombe‐Huntington, J., Jackson, B. P., Grant, M. P., Iskandarani, L., Paganini, C., et al. (2026) · Environmental Research · doi:10.1016/j.envres.2026.124634
Identification and characterisation of microRNAs expressed in articular cartilage and osteoarthritis
Crowe, N. (2014) · UEA Digital Repository (University of East Anglia)
Metadata
- Authors
- Kvist, A. J., Johnson, A. E., Mörgelin, M., Gustafsson, E., Bengtsson, E., Lindblom, K., Aszódi, A., Fässler, R., Sasaki, T., Timpl, R., & Aspberg, A.
- Affiliations
- Lund University (SE)
- Max Planck Society (DE)
- Corresponding author
- Aspberg, Anders
- ORCID
- Venue
- Journal of Biological Chemistry
- Publisher
- Elsevier BV
- Published
- 2006-11
- Volume
- 281
- Issue
- 44
- Pages
- 33127-33139
- ISSN
- 0021-9258 (ISSN-L)
- OpenAlex
- W2072099999
- Cited by
- 102
- References
- 48
- Citations by year
- 2026: 2 · 2024: 4 · 2023: 6 · 2022: 6 · 2021: 4 · 2020: 5 · 2019: 3 · 2018: 4 · 2017: 8 · 2016: 7 · 2015: 3 · 2014: 8 · 2013: 5 · 2012: 4
- FWCI
- 3.18
- Type
- journal article
- Language
- en
- Open access
- hybrid
- doi.org
- Licence
- Locations
- Journal of Biological Chemistry (publishedVersion, cc-by)
- PubMed (publishedVersion)
- Lund University Publications (Lund University) (submittedVersion)
- Research at the University of Copenhagen (University of Copenhagen) (submittedVersion)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Cell Biology › Proteoglycans and glycosaminoglycans research
- Health Sciences › Medicine › Rheumatology › Osteoarthritis Treatment and Mechanisms
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Connective tissue disorders research
- Keywords
- perlecan, chondroitin sulfate E, collagen fibril formation, type II collagen, chondrodysplasia, glycosaminoglycans, chondrocytes, growth plate cartilage, proteoglycans
- MeSH terms
- Animals, Cartilage, Cattle, Cell Line, Cells, Cultured, Chondroitin Sulfates, Glycosaminoglycans, Heparitin Sulfate, Heparan Sulfate, Humans, Protein Binding, Sulfates, Heparan Sulfate Proteoglycans, Chondrocytes, Fibrillar Collagens, Microscopy, Electron, Transmission
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