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, vol.46, no.5, pp.573-579, 2021 (SCI-Expanded) identifier identifier

  • Publication Type: Article / Article
  • Volume: 46 Issue: 5
  • Publication Date: 2021
  • Doi Number: 10.1515/tjb-2021-0048
  • Journal Name: TURKISH JOURNAL OF BIOCHEMISTRY-TURK BIYOKIMYA DERGISI
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus, Academic Search Premier, EMBASE, Food Science & Technology Abstracts, TR DİZİN (ULAKBİM)
  • Page Numbers: pp.573-579
  • Keywords: endoplasmic reticulum stress, leukocytes, phenylalanine, phenylketonuria, OXIDATIVE STRESS, PHENYLALANINE, MUTATIONS, CHOP
  • Ondokuz Mayıs University Affiliated: Yes

Abstract

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.