title: Decidualization is Impaired in Endometrial Stromal Cells from Uterine Rudiments in Mayer-Rokitansky-Küster-Hauser Syndrome.
abstract: OBJECTIVE
  Uterine rudiments from patients with Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS) contain all tissues typically found in the uterus. Endometrium from the rudiments predominantly exhibits basalis-like features, and endometrial proliferative capacity in patients' epithelium and stroma is significantly lower.
  
  
  METHODS
  This single-center, prospective study conducted at a major German university hospital compared in-vitro decidualization in cultured ESCs from MRKHS patients and hysterectomy controls. Primary ESC cultures were established from both sources. Hormone-induced prolactin and IGFBP-1 secretion served as a measure of their ability to undergo decidualization in response to hormonal stimulation. Expression levels of 8 key marker genes of decidualization were also determined.
  
  
  RESULTS
  At day 9, mean secretion of prolactin and IGFBP-1 was significantly reduced by 89.0% and 99.5%, respectively, in MRKHS ESCs vs. hysterectomy controls, both indicating impaired decidualization of MRKHS ESCs. Key decidual markers confirmed impaired decidualization in MRKHS patients.
  
  
  CONCLUSIONS
  Our results indicate that the ESCs from MRKHS patients lack hormone responsiveness as a potential sign of dysfunctional hormone receptor function, which may also play a role in the onset of MRKHS. Further studies are needed to corroborate our findings, directly address receptor function, and elucidate the role of other potential determinants of uterine development and adult function.
authors.forename: Sara Y
authors.initials: SY
authors.lastname: Brucker
authors.forename: Simone
authors.initials: S
authors.lastname: Eisenbeis
authors.forename: Juliana
authors.initials: J
authors.lastname: König
authors.forename: Melanie
authors.initials: M
authors.lastname: Lamy
authors.forename: Madhuri S
authors.initials: MS
authors.lastname: Salker
authors.forename: Ni
authors.initials: N
authors.lastname: Zeng
authors.forename: Harald
authors.initials: H
authors.lastname: Seeger
authors.forename: Melanie
authors.initials: M
authors.lastname: Henes
authors.forename: Dorit
authors.initials: D
authors.lastname: Schöller
authors.forename: Birgitt
authors.initials: B
authors.lastname: Schönfisch
authors.forename: Annette
authors.initials: A
authors.lastname: Staebler
authors.forename: Florin-Andrei
authors.initials: FA
authors.lastname: Taran
authors.forename: Diethelm
authors.initials: D
authors.lastname: Wallwiener
authors.forename: Katharina
authors.initials: K
authors.lastname: Rall
mesh_terms: D058489:46, XX Disorders of Sex Development / Q000378:metabolism / Q000601:surgery; D000293:Adolescent; D000328:Adult; D000013:Congenital Abnormalities / Q000378:metabolism / Q000601:surgery; D004717:Endometrium / Q000002:abnormalities* / Q000378:metabolism / Q000601:surgery; D004958:Estradiol / Q000494:pharmacology; D005260:Female; D015870:Gene Expression / Q000187:drug effects; D006801:Humans; D018970:Insulin-Like Growth Factor Binding Protein 1 / Q000096:biosynthesis / Q000235:genetics; D009095:Mullerian Ducts / Q000002:abnormalities* / Q000378:metabolism / Q000601:surgery; D061251:Primary Cell Culture; D011374:Progesterone / Q000494:pharmacology; D011388:Prolactin / Q000096:biosynthesis / Q000235:genetics; D011446:Prospective Studies; D017154:Stromal Cells / Q000187:drug effects / Q000378:metabolism / Q000473:pathology*; D014621:Vagina / Q000002:abnormalities* / Q000378:metabolism / Q000601:surgery
chemical_list: C032231:IGFBP1 protein, human; D018970:Insulin-Like Growth Factor Binding Protein 1; D011374:Progesterone; D004958:Estradiol; D011388:Prolactin
keywords: Congenital vaginal agenesis; Decidualization; Endometrium; Müllerian agenesis; Müllerian aplasia