Estradiol differentially regulates DUX4, beta-catenin and PAX3/PAX7 in primary myoblasts of facioscapulohumeral muscular dystrophy patients

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HANGÜL C., ÇELİK E. G., KAYA H., EROĞLU O., UYSAL H., KARAÜZÜM S.

TURKISH JOURNAL OF BIOCHEMISTRY-TURK BIYOKIMYA DERGISI, vol.46, no.4, pp.435-444, 2021 (SCI-Expanded) identifier

  • Publication Type: Article / Article
  • Volume: 46 Issue: 4
  • Publication Date: 2021
  • Doi Number: 10.1515/tjb-2020-0351
  • 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.435-444
  • Keywords: beta-catenin, DUX4, estradiol, facioscapulohumeral muscular dystrophy, FSHD, PAX3, PAX7, skeletal muscle, FSHD, D4Z4, EXPRESSION, ESTROGEN, REPEAT, PROGESTERONE, PREGNANCY, APOPTOSIS, LOCUS, WOMEN
  • Akdeniz University Affiliated: Yes

Abstract

Objectives: There is a clinical variability and heterogeneity among Facioscapulohumeral Muscular Dystrophy (FSHD) patients. Escalation after menopause in women, early onset in men suggests that estrogen might be a protective factor on the course of FSHD. In spite of few molecular studies supporting the protective role of estrogen in FSHD in vitro, there is no study revealing the effect of estradiol on the protein levels of DUX4, beta-catenin and PAX3/PAX7. In present study, we investigated the effect of estradiol treatment on the expressions of DUX4, beta-catenin and PAX3/PAX7 protein levels.