Abstract Details

Title New Insights About Myofibrillar Myopathies: The Role Of Metalloproteinases 2 And 9 In The Pathogenesis

Topic Neuromuscular and Clinical Neurophysiology (EMG)

Presentation(s) P2 - Poster Session 2 (5:30 PM-6:30 PM)

Poster/Presentation
Number 12-036

Objective To verify the role of matrix metalloproteinases 2 and 9 (MMPs) in the pathogenesis of myofibrillar myopathies.

Background

There are few reports suggesting that gene expression and activation of various matrix metalloproteinases are deregulated. MMP-2 and MMP-9 represent the two MMPs, which degrade type IV collagen, the component of basement membrane.

Design/Methods

We analyzed the involvement of gelatinases, MMP-2 and MMP-9, in the pathogenesis of myofibrillar myopathy (MFM). Muscle specimens from 23 patients diagnosed with MFM, were immunostained by MMP-2 and MMP-9. We analyzed qualitatively the immunoexpression in three compartments: subsarcolemmal (SSC), intracytoplasmic (ICC) and perinuclear (PNC).

Results 95,7% and 100% samples showed MMP-2 and MMP-9 upregulation ICC, respectively. PNC showed MMP-2 (82,6%) and MMP-9 (8,7%) regulation (p<0.001). SSC and ICC did not present statistical significance. There was no correlation between mutated gene and immunohistochemical pattern distribution

Conclusions

Our results suggest that MMP-2 and/or MMP-9 could participate in the pathomechanism of MFM, causing damage of sarcomere and deposition of protein aggregates.

Authors/Disclosures
Alzira A. Carvalho, MD (FMABC) PRESENTER	No disclosure on file
	No disclosure on file
	No disclosure on file
	No disclosure on file
	No disclosure on file

��ɫ��

��ɫ��