PMID- 10092650
OWN - NLM
STAT- MEDLINE
DCOM- 19990427
LR  - 20190508
IS  - 0021-9258 (Print)
IS  - 0021-9258 (Linking)
VI  - 274
IP  - 14
DP  - 1999 Apr 2
TI  - Fibromodulin-null mice have abnormal collagen fibrils, tissue organization, and
      altered lumican deposition in tendon.
PG  - 9636-47
AB  - Fibromodulin is a member of a family of connective tissue
      glycoproteins/proteoglycans containing leucine-rich repeat motifs. Several
      members of this gene family bind to fibrillar collagens and are believed to
      function in the assembly of the collagen network in connective tissues. Here we
      show that mice lacking a functional fibromodulin gene exhibit an altered
      morphological phenotype in tail tendon with fewer and abnormal collagen fiber
      bundles. In fibromodulin-null animals virtually all collagen fiber bundles are
      disorganized and have an abnormal morphology. Also 10-20% of the bundles in
      heterozygous mice are similar to the abnormal bundles in fibromodulin-null tail
      tendon. Ultrastructural analysis of Achilles tendon from fibromodulin-null mice
      show collagen fibrils with irregular and rough outlines in cross-section.
      Morphometric analysis show that fibromodulin-null mice have on the average
      thinner fibrils than wild type animals as a result of a larger preponderance of
      very thin fibrils in an overall similar range of fibril diameters. Protein and
      RNA analyses show an approximately 4-fold increase in the content of lumican in
      fibromodulin-null as compared with wild type tail tendon, despite a decrease in
      lumican mRNA. These results demonstrate a role for fibromodulin in collagen
      fibrillogenesis and suggest that the orchestrated action of several leucine-rich 
      repeat glycoproteins/proteoglycans influence the architecture of collagen
      matrices.
FAU - Svensson, L
AU  - Svensson L
AD  - Department of Cell and Molecular Biology, University of Lund, P.O. Box 94, S-221 
      00 Lund, Sweden.
FAU - Aszodi, A
AU  - Aszodi A
FAU - Reinholt, F P
AU  - Reinholt FP
FAU - Fassler, R
AU  - Fassler R
FAU - Heinegard, D
AU  - Heinegard D
FAU - Oldberg, A
AU  - Oldberg A
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - J Biol Chem
JT  - The Journal of biological chemistry
JID - 2985121R
RN  - 0 (Carrier Proteins)
RN  - 0 (Chondroitin Sulfate Proteoglycans)
RN  - 0 (Extracellular Matrix Proteins)
RN  - 0 (Fmod protein, mouse)
RN  - 0 (Lum protein, mouse)
RN  - 0 (Lumican)
RN  - 0 (Proteoglycans)
RN  - 0 (RNA, Messenger)
RN  - 126468-95-9 (Fibromodulin)
RN  - 9007-34-5 (Collagen)
RN  - 9056-36-4 (Keratan Sulfate)
SB  - IM
MH  - Animals
MH  - Carrier Proteins/genetics/*physiology
MH  - Cell Line
MH  - Chondroitin Sulfate Proteoglycans/*metabolism
MH  - *Collagen
MH  - Connective Tissue/*abnormalities
MH  - *Extracellular Matrix Proteins
MH  - Fibromodulin
MH  - Immunohistochemistry
MH  - In Situ Hybridization
MH  - Keratan Sulfate/*metabolism
MH  - Lumican
MH  - Mice
MH  - Mice, Knockout
MH  - Microscopy, Electron
MH  - *Proteoglycans
MH  - RNA, Messenger/metabolism
MH  - Tendons/abnormalities/*metabolism
EDAT- 1999/03/27 00:00
MHDA- 1999/03/27 00:01
CRDT- 1999/03/27 00:00
PHST- 1999/03/27 00:00 [pubmed]
PHST- 1999/03/27 00:01 [medline]
PHST- 1999/03/27 00:00 [entrez]
AID - 10.1074/jbc.274.14.9636 [doi]
PST - ppublish
SO  - J Biol Chem. 1999 Apr 2;274(14):9636-47. doi: 10.1074/jbc.274.14.9636.