The effect of APTR, Fn14 and CD133 expressions on liver fibrosis in biliary atresia patients

Pediatr Surg Int. 2020 Jan;36(1):75-79. doi: 10.1007/s00383-019-04582-2. Epub 2019 Sep 23.


Purpose: Although some biomarkers of hepatic progenitor cells have been reported to be involved in the liver fibrosis in patients with biliary atresia (BA), however, research still shows conflicting results. Therefore, we investigated the effect of Alu-mediated p21 transcriptional regulator (APTR), fibroblast growth factor-inducible 14 (Fn14) and CD133 expressions on liver fibrosis in Indonesian BA patients.

Methods: Nineteen liver samples from BA patients and 9 liver specimens from non-BA controls were obtained. The expressions of APTR, Fn14 and CD133 were analyzed by quantitative real-time polymerase chain reaction (qPCR).

Results: APTR expression was strongly up-regulated (1.5-fold) in liver BA specimens compared to liver controls (ΔCT 3.2 ± 0.6 vs 3.8 ± 0.51; p = 0.028). Moreover, Fn14 and CD133 expressions were similar in the BA and control groups (ΔCT 2.7 ± 1.3 vs. 1.4 ± 1.6, p = 0.07; and 12.0 ± 3.7 vs. 11.78 ± 2.30, p = 0.88, respectively). Intriguingly, CD133 expression was strongly related with the survival of BA patients (p = 0.0061), but not with age at Kasai procedure (p = 0.36) and the presence of cirrhosis (p = 0.77).

Conclusion: We present the first study of aberrant APTR expressions in the liver of BA infants which might contribute to liver fibrogenesis in BA infants.

Keywords: APTR; Aberrant expression; Biliary atresia; Indonesia; Liver fibrosis; qPCR.

MeSH terms

  • AC133 Antigen / metabolism*
  • Biliary Atresia / metabolism*
  • Biliary Atresia / surgery
  • Biomarkers / metabolism
  • Case-Control Studies
  • Female
  • Humans
  • Infant
  • Liver / metabolism*
  • Liver Cirrhosis / metabolism*
  • Male
  • RNA, Long Noncoding / metabolism*
  • Real-Time Polymerase Chain Reaction
  • TWEAK Receptor / metabolism*
  • Up-Regulation


  • AC133 Antigen
  • Biomarkers
  • PROM1 protein, human
  • RNA, Long Noncoding
  • TNFRSF12A protein, human
  • TWEAK Receptor
  • long non-coding RNA APTR, human