Serebral palsili çocuklarda ubiqinitasyon sistemindeki değişiklikler

Öz

Amaç: Serebral palsili (SP) çocuklarda ubikitinasyon süreçleri ile ilişkili periferik kanda Ubiquitin Karboksi Terminal Hidrolaz-L1 enzimi (UHC-L1), Transaktif Yanıt DNA Bağlayıcı Protein-43 (TDP-43) ve Cullin-3 düzeylerini araştırmayı amaçladık. . Materyal ve Metod: Birinci grubuna SP'li 50 hasta dahil edildi. Kontrol grubunda yaş ve cinsiyet açısından hasta grupları ile eşleşen 30 sağlıklı çocuk vardı. SP, SP tipi, botoks uygulaması, ortez kullanımı, annenin doğum yaşı ve ek sorunlar için risk faktörlerini kayddildi. Bu çalışmaya 6-10 yaş arası, SP tanısı almış, genetik, metabolik hastalık veya mental retardasyon öyküsü olmayan hastalar dahil edildi. Bulgular: SP grubunda 32 kadın ve 18 erkek hasta bulunurken, kontrol grubunda 19 kadın ve 11 erkek gönüllü vardı. Anne yaşı SP grubunda anlamlı olarak yüksekti (p=0,002). Çalışmamızda kontrol grubu ile SP grubunun UCH-L1, TDP-43 ve Cullin 3 düzeyleri açısından karşılaştırılması sonucunda; SP grubunda UCH-L1 (p=0,048), TDP-43 (p=0,028) ve Cullin 3 (p=0,042) düzeyleri kontrol grubuna göre istatistiksel olarak anlamlı düşük bulundu. Sonuç: SP grubundaki UCHL-L1, Cullin 3 ve TDP-43 moleküllerinin düşük serum konsantrasyonları ve bu moleküllerin birbirleriyle istatistiksel olarak pozitif korelasyonu, ubiquitination sisteminin bozulmasından sonra nöronal patofizyolojinin anlaşılmasına yardımcı olabilir.

Anahtar Kelimeler

• Ubiquitin Karboksi Terminal Hidrolaz-L1 enzimi
• Transaktif Yanıt DNA Bağlayıcı Protein-43
• Cullin-3
• serebral palsi

Changes in the ubiquitination system in children with cerebral palsy

Öz

Objective: We aimed to investigate the levels of Ubiquitin Carboxy Terminal Hydrolase-L1 enzyme (UHC-L1), Transactive Response DNA Binding Protein-43 (TDP-43) and Cullin-3 in peripheral blood associated with ubiquitination processes in children with cerebral palsy (CP). Materials and Methods: We included 50 children with CP in the first patient group. In the control group, there were 30 healthy children who were matched with the patient groups in terms of age and gender. We also recorded risk factors for CP, CP type, botox application, orthosis use, maternal age at birth, and additional problems. Patients aged 6-10 years, diagnosed with CP, without genetic, metabolic disease or mental retardation history were included in this study. Results: There were 32 female and 18 male patients in the CP group, while there were 19 female and 11 male volunteers in the control group. Maternal age was significantly higher in the CP group (p=0.002). In our study, as a result of the comparison between the control group and the CP group in terms of UCH-L1, TDP-43 and Cullin 3 levels; the levels of UCH-L1 (p=0.048), TDP-43 (p=0.028) and Cullin 3 (p=0.042) in the CP group were found to be statistically significantly lower than the levels of the control group. Conclusion: The low serum concentrations of UCHL-L1, Cullin 3 and TDP-43 molecules in the CP group and the statistically positive correlation of these molecules with each other may help to understand the neuronal pathophysiology after disruption of the ubiquitination system.

Anahtar Kelimeler

• Ubiquitin Carboxy Terminal Hydrolase-L1 enzyme
• Transactive Response DNA Binding Protein-43
• Cullin-3
• cerebral palsy

Kaynakça

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