Thiol/Disulfide Balance in Induced Phenylketonuria Model

Abstract

Amaç: Fenilketonüri (PKU), fenilalanin hidroksilaz enzim eksikliği ile karakterize nadir görülen bir kalıtsal metabolik hastalıktır. Bu enzimin eksikliği, kan fenilalanin seviyesini yükselterek, beyinde fenilalanin birikmesine ve geri dönüşümsüz nörolojik hasar oluşmasına neden olur. . Bu çalışmada fenilketonüri modelinde beyin tiyol/disülfit dengesindeki değişimin belirlenmesi amaçlanmıştır. Yöntem: PKU modeli oluşturulmuş sıçan yavrularında (n:7) ve kontrol grubunda beyin total tiyol ve serbest tiyol seviyesi modifiye elman yöntemi ile ölçüldü. Total tiyol ve native tiyol seviyelerine göre disülfit seviyesi hesaplandı. Bulgular: PKU grubun beyin total tiyol seviyesi kontrol grubuna göre istatistiksel olarak azdır (*p=0.0369). PKU grubun beyin serbest tiyol seviyesi kontrol grubuna göre istatistiksel olarak azdır (****p<0.001). PKU grubun beyin disülfit seviyesi kontrol grubuna göre istatistiksel olarak değişiklik göstermemiştir (p=0.1107). Sonuç: PKU’de oluşan oksidatif strese tiyol/disülfit seviyelerininde değişikliklerinde etki edebileceği sonucuna varılmıştır. Literatürde ilk kez rapor edilen çalışmada PKU patofizyolojine tiyol/disülfit dengesininde değişiklik olduğu gösterilmiştir.

Keywords

• Fenilketonüri Modeli
• Tiyol/Disülfit Dengesi
• Oksidatif Stres
• Beyin

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