Phenotypic effects of biglycan deficiency are linked to collagen fibril abnormalities, are synergized by decorin deficiency, and mimic Ehlers-Danlos-like changes in bone and other connective tissues

Journal of Bone and Mineral Research

Volumn 17, Issue 7, 2002, Pages 1180-1189

  Corsi, A ^a   Xu, T ^b   Chen, X D ^b   Boyde, A ^c   Liang, J ^b   Mankani, M ^b   Sommer, B ^b   Iozzo, R V ^d   Eichstetter, I ^d   Robey, P Gehron ^b   Bianco, P ^e   Young, M F ^b  

^a Università dell quila   (Italy)

^b NATIONAL INSTITUTES OF HEALTH   (United States)

^c UNIVERSITY COLLEGE LONDON   (United Kingdom)

^d THOMAS JEFFERSON UNIVERSITY   (United States)

^e SAPIENZA UNIVERSITY OF ROME   (Italy)

Author keywords

Biglycan; Collagen fibrils; Decorin; Double knockout; Ehlers Danlos

Indexed keywords

BIGLYCAN; COLLAGEN FIBRIL; DECORIN; GLYCOSAMINOGLYCAN; PROTEOGLYCAN; COLLAGEN; SCLEROPROTEIN;

ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; BONE; BONE MATRIX; BONE STRUCTURE; CELL ULTRASTRUCTURE; COLLAGEN DEFECT; CONTROLLED STUDY; CUTANEOUS PARAMETERS; DERMIS; DISEASE SEVERITY; EHLERS DANLOS SYNDROME; GENE DISRUPTION; HISTOPATHOLOGY; KNOCKOUT MOUSE; MALE; MOUSE; NONHUMAN; NUCLEOTIDE SEQUENCE; ORGAN DISTRIBUTION; OSTEOPENIA; PHENOTYPE; PROGERIA; PROTEIN BINDING; PROTEIN DEFICIENCY; PROTEIN EXPRESSION; PROTEIN FAMILY; SUBCUTANEOUS TISSUE; TENDON; ANIMAL; CONNECTIVE TISSUE; GENETICS; METABOLISM; MOUSE MUTANT; PATHOLOGY; PHYSIOLOGY; SKIN;

ANIMAL; BONE AND BONES; COLLAGEN; CONNECTIVE TISSUE; EHLERS-DANLOS SYNDROME; MALE; MICE; MICE, KNOCKOUT; PHENOTYPE; PROTEOGLYCANS; SKIN; SUPPORT, NON-U.S. GOV'T; ANIMALS; EXTRACELLULAR MATRIX PROTEINS;

EID: 0035991242     PISSN: 08840431     EISSN: None     Source Type: Journal    
DOI: 10.1359/jbmr.2002.17.7.1180     Document Type: Article

References (41)

1. Proteoglycans of developing bone
2. Deduced protein sequence of bone small proteoglycan I (biglycan) shows homology with proteoglycan II (decorin) and several nonconnective tissue proteins in a variety of species
3. Leucine-rich repeat glycoproteins of the extracellular matrix
4. Matrix proteoglycans. From molecular design to cellular function
5. The X-chromosomal human biglycan gene BGN is subject to X inactivation but is transcribed like an X-Y homologous gene
6. Selective inactivity of TGF-beta/decorin complexes
7. Proteoglycans in cell regulation
8. The biology of the small leucine-rich proteoglycans. Functional network of interactive proteins
9. The effect of proteoglycans on the morphology of collagen fibrils formed in vitro
10. Binding of fibromodulin and decorin to separate sites on fibrillar collagens
11. Regulation of corneal collagen fibrillogenesis in vitro by corneal proteoglycan (lumican and decorin) core proteins
12. Biosynthesis and interactions of small chondroitin/dermatan sulphate proteoglycans
13. Decorin-type I collagen interaction. Presence of separate core protein-binding domains
14. Interaction of biglycan with type I collagen
15. Collagen fibril formation
16. Increased diameters of collagen fibrils precipitated in vitro in the presence of decorin from various connective tissues
17. Expression and localization of the two small proteoglycans biglycan and decorin in developing human skeletal and non-skeletal tissues
18. Targeted disruption of decorin leads to abnormal collagen fibril morphology and skin fragility
19. Lumican regulates collagen fibril assembly: Skin fragility and corneal opacity in the absence of lumican
20. Targeted disruption of the biglycan gene leads to an osteoporosis-like phenotype in mice
21. Basic and "special" stains for plastic sections in bone marrow histopathology, with special reference to May-Grunwald Giemsa and enzyme histochemistry
22. Osteoconduction in large macroporous hydroxyapatite ceramic implants: Evidence for a complementary integration and disintegration mechanism
23. Standards for mineral quantitation of human bone by analysis of backscattered electron images
24. Mean atomic number and backscattered electron coefficient for some materials with low atomic mean number
25. Monte Carlo simulation of electron backscattering from compounds with low mean atomic number
26. Structural abnormalities in the dermal collagen and elastic matrix from the skin of patients with inherited connective tissue disorders
27. Dermal changes in Ehlers-Danlos syndrome
28. The Ehlers-Danlos syndrome
29. Differential ultrastructural aberrations of collagen fibrils in Ehlers-Danlos syndrome types I-IV as a means of diagnostics and classification
30. The Ehlers-Danlos syndrome: On beyond collagens
31. An inherited connective tissue disease in the horse
32. Abnormalities of collagen fibrils in a rabbit with a connective tissue defect similar to Ehlers-Danlos syndrome
33. Collagen fibrillogenesis in vitro: Interaction of types I and V collagen regulates fibril diameter
34. Type I and type III collagen interactions during fibrillogenesis
35. Fibromodulin-null mice have abnormal collagen fibrils, tissue organization, and altered lumican deposition in tendon
36. Differential expression of lumican and fibromodulin regulate collagen fibrillogenesis in developing mouse tendons
37. Ehlers-Danlos syndromes revised nosology, Villefranche, 1997
38. Biglycan and fibromodulin double knockout mice develop ectopic sesamoid bones and premature osteoarthritis in the knee joint
39. Assessment of bone in Ehlers Danlos syndrome by ultrasound and densitometry
40. Glycosaminoglycan-free small proteoglycan core protein is secreted by fibroblasts from a patient with a syndrome resembling progeroid
41. A genetic defect in the byosinthesis of dermatan sulfate proteoglycan. Galactosyltransferase I deficiency in fibroblasts from a patient with a progeroid syndrome