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Collagen gene cluster expression and liver fibrogenesis in patients with biliary atresia: a preliminary study

OBJECTIVE: Biliary atresia (BA) is a progressive fibro-obliterative disease of the biliary tract, which results in end-stage liver disease. However, liver fibrosis progression may continue even after Kasai surgery. Recent evidence showed that collagen plays a pivotal role in the progression of liver...

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Detalles Bibliográficos
Autores principales: Gunadi, Puspitarani, Dyah Ayu, Vujira, Khanza Adzkia, Utami, Fadila Dyah Trie, Devana, Edita Mayda, Halim, Fiqih Vidiantoro, Iskandar, Kristy, Makhmudi, Akhmad
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10690962/
https://www.ncbi.nlm.nih.gov/pubmed/38041174
http://dx.doi.org/10.1186/s13104-023-06636-0
Descripción
Sumario:OBJECTIVE: Biliary atresia (BA) is a progressive fibro-obliterative disease of the biliary tract, which results in end-stage liver disease. However, liver fibrosis progression may continue even after Kasai surgery. Recent evidence showed that collagen plays a pivotal role in the progression of liver fibrosis in BA. However, most studies were conducted in developed countries. We investigated the expressions of the collagen gene cluster (COL6A1, COL6A2, COL6A3, and COL1A1) in BA patients in Indonesia. RESULTS: There was a significant down-regulated expression of COL6A1 (ΔC(T) 9.06 ± 2.64 vs. 5.42 ± 2.41; p = 0.0009), COL6A2 (ΔC(T) 8.25 ± 2.07 vs. 5.77 ± 3.51; p = 0.02), COL6A3 (ΔC(T) 11.2 ± 6.08 vs. 6.78 ± 3.51; p = 0.024), and COL1A1 (ΔC(T) 3.26 ± 1.71 vs. 0.19 ± 2.76; p = 0.0015) in BA patients compared to controls. Interestingly, the collagen gene cluster expressions were significantly associated with the presence of cirrhosis (p = 0.0085, 0.04, and 0.0283 for COL6A1, COL6A2, and COL6A3, respectively). In conclusion, our study shows the changes in the collagen gene cluster, particularly collagen type I and VI, expressions in patients with BA in a particular developing country. Our findings suggest the role of these collagen gene clusters in the liver fibrogenesis of BA. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1186/s13104-023-06636-0.