No 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

Introduction: Nitric oxide (NO), hydrogen sulfide (H2S), and carbon monoxide (CO) are known as gaseous autocoids. It is not clear how the application of exogenous NO, H2S and CO alters the thiol/disulfide balance and advanced oxidation protein products (AOPPs) in the hippocampus and serum. Materials and Methods: In the study, rats were exogenously injected with L-arginine (100 mg/kg) as a NO donor, NaHS (10 mg/ kg) as a H2S donor, and CORM-2 (10 mg/kg) as a CO donor (II) (a tricarbonyldichlororuthenium dimer). Thiol/disulfide balance and advanced protein oxidation products were analyzed in hippocampus and serum samples. Results: The native thiol level in the hippocampus of the L-arginine group was statistically decreased compared to the native thiol level of the control group (p≤0.0001). The disulfide level in the hippocampus of the L-arginine group was statistically increased compared to the control group (p=0.009). Hippocampal total thiol level of NaHS group and CORM-2 group increased statistically (p=0.008,p=0.0157, respectively), while serum disulfide level of CORM-2 group decreased (p=0.0005). Serum and hippocampus AOPPs levels of the NaHS group were statistically increased compared to the control group (***p=0.0006, **p=0.0047, respectively). Similarly, the hippocampal AOPP level in the CORM-2 group was found to be statistically increased compared to the AOPP level in the control group (p=0.0437). Conclusion: As NaHS can improve thiol/disulfide balance, new studies are needed for CORM-2 and L-Arginine. This study is the first to report the effects of NO, H2S and CO on thiol/disulfide balance and AOPPs in the hippocampus and serum. Keywords: NO, H2S, CO, AOPPs, Thiol/disulfide