Banded aggregates containing fibrillin are present in the cartilage matrix adjacent to chondrocytes in individuals affected with scoliosis

1992 ◽  
Vol 50 (1) ◽  
pp. 892-893
Author(s):  
Douglas R. Keene ◽  
Magaret Fairhurst ◽  
Catherine C. Ridgway ◽  
Lynn Y. Sakai
Keyword(s):  
Connective Tissue ◽  
Marfan Syndrome ◽  
Cross Section ◽  
Immunoelectron Microscopy ◽  
Cartilage Matrix ◽  
Negative Stain ◽  
Gene Coding ◽  
Rotary Shadowing

Matrix microfibrils are present in the connective tissue matrices of all tissues. Following standard TEM processing, they appear in cross section as cylindrical fibrils 8-10 nm in diameter, often associated with amorphous elastin. They are also seen in the absence of amorphous elastin, for example in the shallow papillary layer of skin, and also in cartilage matrix (Figure 1). Negative stain and rotary shadowing studies suggest that microfibrils are composed of laterally associated globular structures connected by fine filamentous strands (“ beaded strings”), and that they are extendable. Immunoelectron microscopy has demonstrated that fibrillin, a 350 Kd glycoprotein, is distributed along all microfibrils with a relaxed periodicity of about 54 nm The gene coding for fibrillin has recently been identified and is defective in the Marfan syndrome.

scholarly journals A Case Report of Marfan Syndrome with Literature Review

2015 ◽  
Vol 4 (4) ◽  
pp. 269
Author(s):  
Hemanth Kumar Kalla ◽  
Swarna Kumari ◽  
CH Rama rao ◽  
MKR Parthasarathy ◽  
S Surya prakash Reddy ◽  
...  
Keyword(s):  
Case Report ◽  
Connective Tissue ◽  
Marfan Syndrome ◽  
Clinical Signs ◽  
Connective Tissue Disorder ◽  
Multiple Organ ◽  
Review Of The Literature ◽  
Organ Systems ◽  
Loin Pain ◽  
Routine Physical Examination

Marfan syndrome(MFS) is a connective tissue disorder that affects multiple organ systems. Cardiovascular, ocular, and skeletal abnormalities are cardinal features of the syndrome. Its incidence is among the highest of any heritable disorder.Most patients who have Marfan syndrome are usually diagnosed incidentally when they present for a routine physical examination for various reasons. The purpose of this paper is to provide a review of the literature, as well as describe a 22-year-old male with MFS and right hydroureteronephrosis diagnosed incidentally when he attended our hospital for complaints of fever and right loin pain. This case report emphasizes importance of “Revised Ghent criteria” for the diagnosis of MFS and highlights various clinical signs of MFS<strong>.</strong>

A Preliminary Study on the Angular Distribution of Scattered Ultrasound from Bovine Liver and Myocardium

1987 ◽  
Vol 9 (2) ◽  
pp. 132-145
Author(s):  
Thomas M. Burke ◽  
Ernest L. Madsen ◽  
James A. Zagzebski
Keyword(s):  
Angular Distribution ◽  
Connective Tissue ◽  
Cross Section ◽  
Unit Volume ◽  
Bovine Liver ◽  
Experimental Conditions ◽  
Ultrasonic Scattering ◽  
Differential Scattering Cross Section ◽  
Scattering Cross ◽  
Preliminary Study

Measurements were performed on freshly-excised bovine liver and myocardium to determine the ultrasonic scattering nature of the tissues under a variety of experimental conditions. Results for the angular distribution of the differential scattering cross section per unit volume of tissue are reported for scattering angles spanning 170 to 44 degrees for interrogating frequencies of 1.0, 2.25, 3.5 and 5.0 MHz. Fresh and aged tissues, some with abnormally high connective tissue content were analyzed. The results are compared to previously-published works.

The Role of Soft Connective Tissue in the Response of Pig Articular Cartilage in Organ Culture to Excess of Retinol

1973 ◽  
Vol 13 (1) ◽  
pp. 205-219
Author(s):  
M. E. J. BARRATT
Keyword(s):  
Articular Cartilage ◽  
Connective Tissue ◽  
Organ Culture ◽  
Growing Pigs ◽  
Cartilage Matrix ◽  
Normal Medium ◽  
Chondroid Tissue ◽  
Extensive Degradation

The action of excess retinol on articular cartilage from growing pigs was studied in organ culture. Retinol had little or no effect on explants of articular cartilage alone, but if the explants were cut so as to include some of the marrow tissue in the invasion cavities, or were cultivated near or in contact with capsular tissue, retinol caused extensive degradation of the cartilage matrix, as indicated by loss of metachromatic staining properties. Many chondrocytes were released from their capsules and assumed a fibroblast-like form. Two types of regeneration were seen. In control explants that included part of the invasion zone, cells below the explant laid down a metachromatic matrix; in similar explants cultured in the presence of retinol, a non-metachromatic osteoid-like tissue was formed at this site. There was little recovery when retinol-treated explants were transferred to normal medium, although both osteoid and chondroid tissue were sometimes regenerated.

scholarly journals Dural ectasia in Marfan syndrome and other hereditary connective tissue disorders: a 10-year follow-up study

2019 ◽  
Vol 19 (8) ◽  
pp. 1412-1421 ◽  
Author(s):  
Tordis Böker ◽  
Thy Thy Vanem ◽  
Are Hugo Pripp ◽  
Svend Rand-Hendriksen ◽  
Benedicte Paus ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Marfan Syndrome ◽  
Connective Tissue Disorders ◽  
Dural Ectasia ◽  
Follow Up Study

A Case Report of Marfan Syndrome with Literature Review

2015 ◽  
Vol 4 (4) ◽  
pp. 269
Author(s):  
Hemanth Kumar Kalla ◽  
Swarna Kumari ◽  
CH Rama rao ◽  
MKR Parthasarathy ◽  
S Surya prakash Reddy ◽  
...  
Keyword(s):  
Case Report ◽  
Connective Tissue ◽  
Marfan Syndrome ◽  
Clinical Signs ◽  
Connective Tissue Disorder ◽  
Multiple Organ ◽  
Review Of The Literature ◽  
Organ Systems ◽  
Loin Pain ◽  
Routine Physical Examination

Marfan syndrome(MFS) is a connective tissue disorder that affects multiple organ systems. Cardiovascular, ocular, and skeletal abnormalities are cardinal features of the syndrome. Its incidence is among the highest of any heritable disorder.Most patients who have Marfan syndrome are usually diagnosed incidentally when they present for a routine physical examination for various reasons. The purpose of this paper is to provide a review of the literature, as well as describe a 22-year-old male with MFS and right hydroureteronephrosis diagnosed incidentally when he attended our hospital for complaints of fever and right loin pain. This case report emphasizes importance of “Revised Ghent criteria” for the diagnosis of MFS and highlights various clinical signs of MFS<strong>.</strong>

scholarly journals Microfibril-associated glycoprotein-1 (MAGP-1) is specifically located on the beads of the beaded-filament structure for fibrillin-containing microfibrils as visualized by the rotary shadowing technique.

1996 ◽  
Vol 44 (12) ◽  
pp. 1389-1397 ◽  
Author(s):  
M Henderson ◽  
R Polewski ◽  
J C Fanning ◽  
M A Gibson
Keyword(s):  
Monoclonal Antibody ◽  
Immunoelectron Microscopy ◽  
Narrow Band ◽  
Immunogold Labeling ◽  
Structural Role ◽  
Filament Structure ◽  
Fibrillin 1 ◽  
Postembedding Immunoelectron Microscopy ◽  
Microscopic Techniques ◽  
Rotary Shadowing

This study used immunoelectron microscopic techniques to define the ultrastructural location of MAGP-1 on the fibrillin-containing microfibrils of the ocular zonule. A specific anti-MAGP-1 monoclonal antibody (MAb), 11B, was produced that did not crossreact with fibrillin-1 or other microfibrillar proteins. MAb 11B was shown by immunofluorescence to localize intensely to zonular tissue. Postembedding immunoelectron microscopy showed that MAGP-1 was associated with microfibrils throughout the zonule, with the exception of a narrow band of microfibrils at the junction with the lens capsule. With preembedding labeling, the anti-MAGP-1 MAb was found to localize in a crossbanding pattern, at intervals of about 50 nm, to microfibrils throughout the zonule and along bundles of microfibrils in surrounding vitreous tissue. Rotary shadowing of isolated microfibrils showed a "beads on a string" morphology with a periodicity of about 50 nm. With immunogold labeling, the anti-MAGP-1 antibody specifically localized on the beads in a symmetrical manner. Occasionally two gold partides were attached to the same bead, suggesting that multiple MAGP-1 molecules were present in the structure. The results indicate that MAGP-1 is intimately and regularly associated with the bead regions of fibrillin-containing microfibrils. The findings are consistent with a major structural role for MAGP-1 in microfibril biology.

Leptomeningeal cysts in congenital ectopia lentis

1973 ◽  
Vol 38 (5) ◽  
pp. 650-654 ◽  
Author(s):  
Bryce Weir
Keyword(s):  
Connective Tissue ◽  
Marfan Syndrome ◽  
Genetic Disorder ◽  
Ectopia Lentis ◽  
Content Type ◽  
Extradural Cyst ◽  
Common Pathogenesis ◽  
Intrathoracic Meningocele ◽  
Subarachnoid Cyst ◽  
Fine Print

✓ A case of ectopia lentis (Marfan syndrome) with a cerebral subarachnoid cyst, a spinal extradural cyst connected to a lateral intrathoracic meningocele, and perineural cysts is presented. The association of these leptomeningeal cysts in this genetic disorder of connective tissue suggests a common pathogenesis.

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