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Toward an understanding of dural ectasia: a light microscopy study in a murine model of Marfan syndrome
Kevin B Jones1, Loretha Myers, Daniel P Judge
1Department of Orthopaedic Surgery, University of Iowa Hospitals and Clinics, Iowa City, Iowa 52242, USA. kevin-jones@uiowa.edu
Study Design:
Light microscopy study of the lumbar spinal meninges of a murine model of Marfan syndrome.
Objective:
Characterize the pathology of the lumbosacral meninges in Marfan syndrome, seeking clues to the pathophysiology behind dural ectasia.
Summary Of Background Data:
Dural ectasia is common in Marfan syndrome. The etiology of dural ectasia is unknown, but is conjectured to be related to constitutionally weak spinal dura. The morphology of the lumbar dura in Marfan syndrome has not been described, as it has in other tissues affected by Marfan syndrome.
Methods:
The lumbosacral dura were removed from three 4-month-old mice, 1 homozygote (mgR/mgR) expressing the murine Marfan phenotype, 1 heterozygote expressing wild-type phenotype, and 1 homozygote wildtype. Hematoxylin and eosin, elastochrome, and immunohistochemical stains against activated transforming growth factor beta, gelatinase A (matrix metalloproteinase-2), and gelatinase-B (matrix metalloproteinase-9) were used for light microscopic evaluation.
Results:
No difference was noted between the heterozygous and wild-type mice in dural connective tissue morphology. The homozygote (mgR/mgR) had a marked attenuation of the dura overall, in addition to elastic fiber disorganization. The homozygote dura also stained for increased presence of activated transforming growth factor beta and matrix metalloproteinase-2, but not matrix metalloproteinase-9.
Conclusions:
These morphologic findings in the Marfan phenotype mouse mimic the findings of disordered elastic-fibers in other Marfan tissues and demonstrate gross attenuation of the tissue architecture, corroborating the theory that dural ectasia in Marfan syndrome results from hydrostatic pressure on weakened dura. These changes may be due in part to transforming growth factor beta overactivation and gelatinase-A-mediated elastolysis and collagen breakdown.
Insights
Marfan syndrome causes weakened spinal dura, leading to dural ectasia. This study found disorganized elastic fibers and tissue thinning in Marfan mice, supporting the theory of hydrostatic pressure on weakened dura.
Area of Science:
- Connective tissue disorders
- Marfan syndrome pathology
- Spinal dura research
Background:
- Dural ectasia is a common complication of Marfan syndrome.
- The underlying cause of dural ectasia in Marfan syndrome remains unknown.
- Previous research has not detailed the lumbar dura's morphology in Marfan syndrome.
Purpose of the Study:
- To investigate the pathology of the lumbosacral meninges in a murine model of Marfan syndrome.
- To identify potential mechanisms contributing to dural ectasia in Marfan syndrome.
- To describe the morphological characteristics of the dura in Marfan syndrome.
Main Methods:
- Light microscopy was used to examine the lumbar spinal meninges of Marfan syndrome model mice.
- Histological analysis included Hematoxylin and eosin, elastochrome, and immunohistochemical staining.
- Stains targeted activated transforming growth factor beta, matrix metalloproteinase-2, and matrix metalloproteinase-9.
Main Results:
- Marfan phenotype mice (mgR/mgR homozygotes) exhibited overall dural attenuation and disorganized elastic fibers.
- No significant morphological differences were observed in the dura of heterozygote and wild-type mice.
- Homozygote mice showed increased presence of activated transforming growth factor beta and matrix metalloproteinase-2.
Conclusions:
- Morphological findings in Marfan mice mimic other affected tissues, showing disordered elastic fibers and dural attenuation.
- The results support the hypothesis that dural ectasia in Marfan syndrome arises from hydrostatic pressure acting on weakened dura.
- Transforming growth factor beta overactivation and matrix metalloproteinase-2-mediated degradation may contribute to these observed changes.
