Abstract
Proteoglycans (PGs) were investigated in fibroblast cultures from both apparently normal and involved areas of skin from two patients affected with Pseudoxanthoma elasticum (PXE) and compared to control normal cells. Biochemical analysis showed that cells from the PXE-affected patients produced a PG population with stronger polyanion properties, as well as a markedly increased amount of high hydrodynamic-size PGs. Moreover, PGs from PXE-affected cells showed abnormal hydrophobic interaction properties when examined under associative conditions and included heparan sulphate (HS)-containing populations with anomalous electrophoretic mobility. These phenomena were particularly evident in the case of PGs secreted into the growth medium. In agreement with these findings immunohistochemical study showed alterations affecting decorin and biglycan, as well as a different content and distribution of HS-PGs in PXE-affected cells. The same biochemical and morphological alterations were confirmed for both patients on different cell cultures and were present in cells from both apparently normal and affected skin areas, being more pronounced in the latter. Our results indicate that PXE-affected fibroblasts in culture exhibit an abnormal PG metabolism, which could affect the normal assembly of extracellular matrix.
| Original language | English |
|---|---|
| Pages (from-to) | 111-120 |
| Number of pages | 10 |
| Journal | Cell Biochemistry and Function |
| Volume | 14 |
| Issue number | 2 |
| DOIs | |
| Publication status | Published - 1996 |
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Keywords
- Fibroblasts
- Proteoglycans
- Pseudoxanthoma elasticum
- Skin
ASJC Scopus subject areas
- Clinical Biochemistry
- Cell Biology
Cite this
Proteoglycan alterations in skin fibroblast cultures from patients affected with pseudoxanthoma elasticum. / Passi, A.; Albertini, R.; Baccarani Contri, M.; De Luca, G.; De Paepe, A.; Pallavicini, G.; Pasquali Ronchetti, I.; Tiozzo, R.
In: Cell Biochemistry and Function, Vol. 14, No. 2, 1996, p. 111-120.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Proteoglycan alterations in skin fibroblast cultures from patients affected with pseudoxanthoma elasticum
AU - Passi, A.
AU - Albertini, R.
AU - Baccarani Contri, M.
AU - De Luca, G.
AU - De Paepe, A.
AU - Pallavicini, G.
AU - Pasquali Ronchetti, I.
AU - Tiozzo, R.
PY - 1996
Y1 - 1996
N2 - Proteoglycans (PGs) were investigated in fibroblast cultures from both apparently normal and involved areas of skin from two patients affected with Pseudoxanthoma elasticum (PXE) and compared to control normal cells. Biochemical analysis showed that cells from the PXE-affected patients produced a PG population with stronger polyanion properties, as well as a markedly increased amount of high hydrodynamic-size PGs. Moreover, PGs from PXE-affected cells showed abnormal hydrophobic interaction properties when examined under associative conditions and included heparan sulphate (HS)-containing populations with anomalous electrophoretic mobility. These phenomena were particularly evident in the case of PGs secreted into the growth medium. In agreement with these findings immunohistochemical study showed alterations affecting decorin and biglycan, as well as a different content and distribution of HS-PGs in PXE-affected cells. The same biochemical and morphological alterations were confirmed for both patients on different cell cultures and were present in cells from both apparently normal and affected skin areas, being more pronounced in the latter. Our results indicate that PXE-affected fibroblasts in culture exhibit an abnormal PG metabolism, which could affect the normal assembly of extracellular matrix.
AB - Proteoglycans (PGs) were investigated in fibroblast cultures from both apparently normal and involved areas of skin from two patients affected with Pseudoxanthoma elasticum (PXE) and compared to control normal cells. Biochemical analysis showed that cells from the PXE-affected patients produced a PG population with stronger polyanion properties, as well as a markedly increased amount of high hydrodynamic-size PGs. Moreover, PGs from PXE-affected cells showed abnormal hydrophobic interaction properties when examined under associative conditions and included heparan sulphate (HS)-containing populations with anomalous electrophoretic mobility. These phenomena were particularly evident in the case of PGs secreted into the growth medium. In agreement with these findings immunohistochemical study showed alterations affecting decorin and biglycan, as well as a different content and distribution of HS-PGs in PXE-affected cells. The same biochemical and morphological alterations were confirmed for both patients on different cell cultures and were present in cells from both apparently normal and affected skin areas, being more pronounced in the latter. Our results indicate that PXE-affected fibroblasts in culture exhibit an abnormal PG metabolism, which could affect the normal assembly of extracellular matrix.
KW - Fibroblasts
KW - Proteoglycans
KW - Pseudoxanthoma elasticum
KW - Skin
UR - http://www.scopus.com/inward/record.url?scp=0029938207&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0029938207&partnerID=8YFLogxK
U2 - 10.1002/(SICI)1099-0844(199606)14:2<111::AID-CBF653>3.0.CO;2-#
DO - 10.1002/(SICI)1099-0844(199606)14:2<111::AID-CBF653>3.0.CO;2-#
M3 - Article
C2 - 8640951
AN - SCOPUS:0029938207
VL - 14
SP - 111
EP - 120
JO - Cell Biochemistry and Function
JF - Cell Biochemistry and Function
SN - 0263-6484
IS - 2
ER -