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Alport Sendromu

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<strong>Alport</strong> <strong>Sendromu</strong> – Patogenez<br />

• <strong>Alport</strong> <strong>Sendromu</strong>nda podositlerden TGF-1 yapımı artar<br />

• TGF-1 fibrozisi ve ekstraselüler matriks birikimini artırır<br />

• TGF-1 blokajı ile GBMda fokal matriks birikimi azalır,<br />

ama ayaksı çıkıntıların düzleşmesi düzelmez<br />

• Preklinik test aşaması: TGF-1 ve alfa1 integrin blokjı ile GBM da<br />

anormal laminin birikimini önleyerek proteinüriyi azaltır<br />

Cosgrove D ve ark. Integrin alpha1beta1 and Transforming Growth Factorbeta1<br />

Play Distinct Roles in <strong>Alport</strong> Glomerular Pathogenesis and Serve as Dual<br />

Targets for Metabolic Therapy. American Journal of Pathology, Vol. 157, No.<br />

5, November 2000

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