STRUCTURE AND FUNCTION OF CONNECTIVE TISSUE PROTEOGLYCANS

Author(s):  
J.R. Hassell ◽  
W.E. Horton ◽  
D.M. Noonan ◽  
K.J. Doege ◽  
G.W. Laurie
2005 ◽  
Vol 22 (2) ◽  
pp. 244-256 ◽  
Author(s):  
Christina M. Consolino ◽  
Franck Duclos ◽  
Jane Lee ◽  
Roger A. Williamson ◽  
Kevin P. Campbell ◽  
...  

α-Sarcoglycan-deficient ( Sgca-null) mice provide potential for elucidating the pathogenesis of limb girdle muscular dystrophy type 2D (LGMD 2D) as well as for studying the effectiveness of therapeutic strategies. Skeletal muscles of Sgca-null mice demonstrate an early onset of extensive fiber necrosis, degeneration, and regeneration, but the progression of the pathology and the effects on muscle structure and function throughout the life span are not known. Thus the phenotypic accuracy of the Sgca-null mouse as a model of LGMD 2D has not been fully established. To investigate skeletal muscle structure and function in the absence of α-sarcoglycan throughout the life span, we analyzed extensor digitorum longus and soleus muscles of male and female Sgca-null and wild-type mice at 3, 6, 12, and 18 mo of age. Maximum isometric forces and powers were measured in vitro at 25°C. Also determined were individual myofiber cross-sectional areas and numbers, water content, and the proportion of the cross section occupied by connective tissue. Muscle masses were 40–100% larger for Sgca-null compared with age- and gender-matched wild-type mice, with the majority of the increased muscle mass for Sgca-null mice attributable to greater connective tissue and water contents. Although the greater mass of muscles in Sgca-null mice was primarily noncontractile material, absolute forces and powers were maintained near control levels at all ages, indicating a successful adaptation to the deficiency in α-sarcoglycan not observed at any age in LGMD 2D patients.


1986 ◽  
Vol 61 (1) ◽  
pp. 312-317 ◽  
Author(s):  
J. C. Van Oostdam ◽  
D. C. Walker ◽  
K. Knudson ◽  
P. Dirks ◽  
R. W. Dahlby ◽  
...  

We compared the effect of breathing dry air (0.70 mg H2O/l) with that of breathing room air (8.62 mg H2O/l) in guinea pigs anesthetized with urethane. The data showed that breathing dry air caused a reduction of extravascular water (EVW) in the trachea (P less than 0.01) but not the lung. Structural analysis showed that this water loss occurred from the loose connective tissue of the submucosa. Histamine dose response curves performed on the animals showed that breathing dry air caused an increase in the maximum response (delta max RL) (P less than 0.01) without changing either the dose required to produce 50% of the delta max RL or the ratio of delta max RL to this dose. We conclude that breathing dry air produces an acute reduction of EVW of the loose connective tissue of the airways and an increase in the maximum response to histamine.


Author(s):  
Kuen-Shan Hung ◽  
M. Sue Hertweck ◽  
John D. Hardy ◽  
Clayton G. Loosli

A small number of fibroblasts (alveolar septal cells, pulmonary interstitial cells, mesenchymal cells) are present in the alveolar areas of the adult lungs. These cells, like fibroblasts elsewhere, may be important in production and maintenance of connective tissue fibers found throughout the lung. Very little attention has been given to these cells in studies of the lungs. Therefore their precise structure and function are not clear. This report demonstrates filaments in the fibroblasts in the alveolar ducts and alveolar walls of the mouse lungs.The lungs were inflated with 2% glutaraldehyde, post-fixed with 1% osmium tetroxide and processed for electron microscopy. The cell body and processes of the alveolar fibroblast are located in the interalveolar septum (Fig. I).


Author(s):  
Z. V. Nesterenko

Review article on the problem of celiac disease, which is widespread with diverse range of clinical manifestations and inadequate diagnosis, is presented. The conciderable prevalence of connective tissue disorders in the pediatric population, the important role of connective tissue in the function of all organ systems, including the gastrointestinal system, necessitates studying the impact of the impaired structure and function of connective tissue on the development of celiac symptoms. The paper describes the historical development of the concept of “celiac disease”, the process of studying the pattern of the onset and development of symptoms of the disease; modern studies explaining the varied complex mechanisms of the disease.The article cites the researchers who studied the role of connective tissue in the structure and function of all body systems with identifying the impact of connective tissue disorders on the development of the gastrointestinal pathology (abnormal motor- tonic activity, reflux disease, dysbiosis, disturbance of autonomic homeostasis) and the manifestation of celiac disease associated with connective tissue disorders.The conclusion stresses the need to consider the problem of celiac disease as a manifestation of the pathology of the whole organism with the obligatory diagnosis of comorbid diseases, including those associated with connective tissue disorders, which will provide a more successful therapy for celiac disease and an improved prognosis.


2014 ◽  
Vol 155 (12) ◽  
pp. 453-460 ◽  
Author(s):  
Lajos Jakab

The author summarizes the structure of the connective tissues, the increasing motion of the constituents, which determine the role in establishing the structure and function of that. The structure and function of the connective tissue are related to each other in the resting as well as inflammatory states. It is emphasized that cellular events in the connective tissue are part of the defence of the organism, the localisation of the damage and, if possible, the maintenance of restitutio ad integrum. The organism responds to damage with inflammation, the non specific immune response, as well as specific, adaptive immunity. These processes are located in the connective tissue. Sterile and pathogenic inflammation are relatively similar processes, but inevitable differences are present, too. Sialic acids and glycoproteins containing sialic acids have important roles, and the role of Siglecs is also highlighted. Also, similarities and differences in damages caused by pathogens and sterile agents are briefly summarized. In addition, the roles of adhesion molecules linked to each other, and the whole event of inflammatory processes are presented. When considering practical consequences it is stressed that the structure (building up) of the organism and the defending function of inflammation both have fundamental importance. Inflammation has a crucial role in maintaining the integrity and the unimpaired somato-psychological state of the organism. Thus, inflammation serves as a tool of organism identical with the natural immune response, inseparably connected with the specific, adaptive immune response. The main events of the inflammatory processes take place in the connective tissue. Orv. Hetil., 2014, 155(12), 453–460.


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