Endoplasmic reticulum stress in leukocytes from phenylketonuric patients

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Bardak K., Özer I., AMANVERMEZ R.

TURKISH JOURNAL OF BIOCHEMISTRY-TURK BIYOKIMYA DERGISI, cilt.46, sa.5, ss.573-579, 2021 (SCI-Expanded) identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 46 Sayı: 5
  • Basım Tarihi: 2021
  • Doi Numarası: 10.1515/tjb-2021-0048
  • Dergi Adı: TURKISH JOURNAL OF BIOCHEMISTRY-TURK BIYOKIMYA DERGISI
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, Academic Search Premier, EMBASE, Food Science & Technology Abstracts, TR DİZİN (ULAKBİM)
  • Sayfa Sayıları: ss.573-579
  • Anahtar Kelimeler: endoplasmic reticulum stress, leukocytes, phenylalanine, phenylketonuria, OXIDATIVE STRESS, PHENYLALANINE, MUTATIONS, CHOP
  • Ondokuz Mayıs Üniversitesi Adresli: Evet

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.