Assessment of dynamic thiol-disulfide homeostasis in patients with lipoid proteinosis (Urbach-Wiethe syndrome)

Creative Commons License

Taskin S., Celik H., Taskin A., Aksoy M., An I., YEŞİLOVA Y.

Revista da Associacao Medica Brasileira, vol.68, no.9, pp.1259-1263, 2022 (SCI-Expanded) identifier identifier

  • Publication Type: Article / Article
  • Volume: 68 Issue: 9
  • Publication Date: 2022
  • Doi Number: 10.1590/1806-9282.20220333
  • Journal Name: Revista da Associacao Medica Brasileira
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus, CAB Abstracts, EMBASE, MEDLINE, Veterinary Science Database, Directory of Open Access Journals
  • Page Numbers: pp.1259-1263
  • Keywords: Disulfides, Lipoid proteinosis, Mucous membrane, Oxidative stress, Thiol
  • Lokman Hekim University Affiliated: No


© 2022 Associacao Medica Brasileira. All rights reserved.OBJECTIVE: Lipoid proteinosis is a rare autosomal recessive genetic dermatological disease that occurs due to the accumulation of hyaline material in the skin and mucous membranes. This study aimed to investigate whether dynamic thiol-disulfide homeostasis is a new marker of oxidative stress in patients suffering from lipoid proteinosis. METHODS: The study group involved 17 patients with lipoid proteinosis and 17 healthy controls with same gender and age. Native thiol, total thiol, disulfide levels, and thiol-disulfide indexes were measured with the fully automated spectrophotometric method described by Erel and Neselioglu, and the results of the two groups were statistically analyzed. RESULTS: Serum total thiol and native thiol levels were significantly lower in lipoid proteinosis group compared to the control group (p=0.020 and p=0.014, respectively). The disulfide levels were found to be higher in lipoid proteinosis group, but there was no significant difference between two groups. CONCLUSIONS: Impaired dynamic thiol-disulfide homeostasis was observed in lipoid proteinosis patients, suggesting that thiol-disulfide homeostasis may have a role in the pathogenesis of this disease.