Defective collagen fibril formation and mineralization in osteogenesis imperfecta with congenital joint contractures (Bruck syndrome)

1993 ◽  
Vol 152 (6) ◽  
pp. 505-508 ◽  
Author(s):  
R. E. Brenner ◽  
U. Vetter ◽  
H. Stöss ◽  
P. K. Müller ◽  
W. M. Teller
Keyword(s):  
Osteogenesis Imperfecta ◽  
Collagen Fibril ◽  
Fibril Formation ◽  
Joint Contractures ◽  
Bruck Syndrome

Osteogenesis imperfecta with joint contractures: Bruck syndrome

1998 ◽  
Vol 28 (2) ◽  
pp. 117-119 ◽  
Author(s):  
M. F. Blacksin ◽  
Beth A. Pletcher ◽  
Miriam David
Keyword(s):  
Osteogenesis Imperfecta ◽  
Joint Contractures ◽  
Bruck Syndrome

scholarly journals Bruck Syndrome: A Rare Disorder in New-Born with Fractures and Contractures

2018 ◽  
Vol 37 (3) ◽  
pp. 276-279
Author(s):  
Suraj Dhaubhadel ◽  
Bimala Baniya ◽  
Hema Joshi ◽  
Ram Hari Chapagain ◽  
Krishna Prasad Paudel
Keyword(s):  
Osteogenesis Imperfecta ◽  
Autosomal Recessive ◽  
Femur Fracture ◽  
Bone Fragility ◽  
Rare Disorder ◽  
Arthrogryposis Multiplex Congenita ◽  
Joint Contractures ◽  
New Born ◽  
Bruck Syndrome

Bruck syndrome is a very rare autosomal recessive syndrome consisting of bone fragility and congenital joint contractures. It is considered as a combination of arthrogryposis multiplex congenita and osteogenesis imperfecta, while some consider it as the autosomal recessive variant of osteogenesis imperfecta. According to the genotype, it has been classified into types 1 and 2. To our knowledge, only about 28 patients of this syndrome have been reported so far worldwide with none been reported from Nepal. Here, we present a patient with generalized osteopenia, bilateral femur fracture and congenital joint contractures of distal extremities.

Osteogenesis imperfecta with congenital joint contractures (Bruck Syndrome)

2008 ◽  
Vol 36 (2) ◽  
pp. 122-126 ◽  
Author(s):  
Denis Viljoen ◽  
Gerry Versfeld ◽  
Peter Beighton
Keyword(s):  
Osteogenesis Imperfecta ◽  
Joint Contractures ◽  
Bruck Syndrome

Ultrastructural analysis of collagen formation in the developing primate amnion

1990 ◽  
Vol 48 (3) ◽  
pp. 106-107
Author(s):  
Barry F. King ◽  
Grete N. Fry
Keyword(s):  
Golgi Apparatus ◽  
Collagen Fibril ◽  
Fibril Formation ◽  
Amniotic Cavity ◽  
Cytological Evidence ◽  
Mammalian Embryo ◽  
Intracellular Location ◽  
Collagen Formation ◽  
Amniotic Epithelium ◽  
Extraembryonic Mesoderm

The amnion surrounding the mammalian embryo consists of the amniotic epithelium facing the amniotic cavity, a layer of extraembryonic mesoderm bordering the exocoelom and an intervening layer of extracellular matrix (Fig. 1). During gestation the amnion expands remarkably to acommodate the rapidly growing embryo. In this study we have examined the process of collagen fibril formation in the developing amnion of the rhesus monkey between 20 and 60 days of gestation.Most cytological evidence of collagen fibril formation was observed in association with the extraembryonic mesodermal cells rather than the amniotic epithelium. The mesodermal cells h ad abundant cisternae of rough endoplasmic reticulum and a prominent Golgi apparatus. Elongated secretory vacuoles were associated with the Golgi apparatus and often contained parallel aggregates of fine filaments (Fig. 2). In some secretory vacuoles, periodic densities also were observed. Some striated collagen fibrils were observed in an apparent intracellular location in long, membrane-limited compartments (Fig. 3). Still other striated fibrils were observed in dense bodies, presumably lysosomes (Fig. 4).

scholarly journals Tyrosine-rich acidic matrix protein (TRAMP) accelerates collagen fibril formation in vitro.

1993 ◽  
Vol 268 (26) ◽  
pp. 19826-19832
Author(s):  
J.R. MacBeath ◽  
D.R. Shackleton ◽  
D.J. Hulmes
Keyword(s):  
Collagen Fibril ◽  
Matrix Protein ◽  
Fibril Formation

scholarly journals Collagen fibril formation. Optimal in vitro conditions and preliminary kinetic results.

1978 ◽  
Vol 253 (18) ◽  
pp. 6578-6585 ◽  
Author(s):  
B.R. Williams ◽  
R.A. Gelman ◽  
D.C. Poppke ◽  
K.A. Piez
Keyword(s):  
Collagen Fibril ◽  
Fibril Formation

scholarly journals Collagen fibril formation during embryogenesis.

1983 ◽  
Vol 80 (11) ◽  
pp. 3354-3358 ◽  
Author(s):  
R. Fleischmajer ◽  
B. R. Olsen ◽  
R. Timpl ◽  
J. S. Perlish ◽  
O. Lovelace
Keyword(s):  
Collagen Fibril ◽  
Fibril Formation

Effect of Oxazolidine E on Collagen Fibril Formation and Stabilization of the Collagen Matrix

2007 ◽  
Vol 55 (17) ◽  
pp. 6813-6822 ◽  
Author(s):  
Santanu Deb Choudhury ◽  
Richard G. Haverkamp ◽  
Samir DasGupta ◽  
Gillian E. Norris
Keyword(s):  
Collagen Fibril ◽  
Collagen Matrix ◽  
Fibril Formation

Description of collagen fibril formation by a theory of polymer crystallization

Biopolymers ◽  
1983 ◽  
Vol 22 (7) ◽  
pp. 1793-1811 ◽  
Author(s):  
Donald G. Wallace ◽  
Andrea Thompson
Keyword(s):  
Collagen Fibril ◽  
Polymer Crystallization ◽  
Fibril Formation
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