Endoplasmic reticulum stress in leukocytes from phenylketonuric patients
   
Yazarlar (3)
Kadriye Bardak
Ondokuz Mayis University, Medical School, Türkiye
Prof. Dr. Işıl ÖZER Ondokuz Mayis University, Medical School, Türkiye
Ramazan Amanvermez Ondokuz Mayis University, Medical School, Türkiye
Makale Türü Açık Erişim Özgün Makale (SSCI, AHCI, SCI, SCI-Exp dergilerinde yayınlanan tam makale)
Dergi Adı Turkish Journal of Biochemistry (Q4)
Dergi ISSN 0250-4685 Wos Dergi Scopus Dergi
Dergi Tarandığı Indeksler SCI-Expanded
Makale Dili İngilizce Basım Tarihi 10-2021
Cilt / Sayı / Sayfa 46 / 5 / 573–579 DOI 10.1515/tjb-2021-0048
Makale Linki online
Özet
Objectives Phenylketonuria (PKU) is a proteinopathy due to the deficiency of phenylalanine hydroxylase (PAH) enzyme. The pathological elevation of phenylalanine (Phe) and its metabolites in PKU is linked to neurological hallmarks and mental disabilities. The aim of this study was to examine the hypothesis that high levels of Phe caused endoplasmic reticulum (ER) stress in PKU patients. Methods We primarily evaluated ER stress markers glucose-regulated protein78 (GRP78) and C/-EBP homologous protein (CHOP), and thiobarbituric acid-reactive substances (TBARS) as a biomarker of oxidative stress in leukocytes and correlated it with blood Phe values from patients with PKU. Patients in this study were selected from individuals who were diagnosed with PKU as a result of the national neonatal screening program and undergone treatment at our university hospital. The subjects were divided into four …
Anahtar Kelimeler
Endoplasmic reticulum stress | Leukocytes | Phenylalanine | Phenylketonuria
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Endoplasmic reticulum stress in leukocytes from phenylketonuric patients

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