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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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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
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author Gunadi
Puspitarani, Dyah Ayu
Vujira, Khanza Adzkia
Utami, Fadila Dyah Trie
Devana, Edita Mayda
Halim, Fiqih Vidiantoro
Iskandar, Kristy
Makhmudi, Akhmad
author_facet Gunadi
Puspitarani, Dyah Ayu
Vujira, Khanza Adzkia
Utami, Fadila Dyah Trie
Devana, Edita Mayda
Halim, Fiqih Vidiantoro
Iskandar, Kristy
Makhmudi, Akhmad
author_sort Gunadi
collection PubMed
description 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.
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spelling pubmed-106909622023-12-02 Collagen gene cluster expression and liver fibrogenesis in patients with biliary atresia: a preliminary study Gunadi Puspitarani, Dyah Ayu Vujira, Khanza Adzkia Utami, Fadila Dyah Trie Devana, Edita Mayda Halim, Fiqih Vidiantoro Iskandar, Kristy Makhmudi, Akhmad BMC Res Notes Research Note 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. BioMed Central 2023-12-01 /pmc/articles/PMC10690962/ /pubmed/38041174 http://dx.doi.org/10.1186/s13104-023-06636-0 Text en © The Author(s) 2023 https://creativecommons.org/licenses/by/4.0/Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) . The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/ (https://creativecommons.org/publicdomain/zero/1.0/) ) applies to the data made available in this article, unless otherwise stated in a credit line to the data.
spellingShingle Research Note
Gunadi
Puspitarani, Dyah Ayu
Vujira, Khanza Adzkia
Utami, Fadila Dyah Trie
Devana, Edita Mayda
Halim, Fiqih Vidiantoro
Iskandar, Kristy
Makhmudi, Akhmad
Collagen gene cluster expression and liver fibrogenesis in patients with biliary atresia: a preliminary study
title Collagen gene cluster expression and liver fibrogenesis in patients with biliary atresia: a preliminary study
title_full Collagen gene cluster expression and liver fibrogenesis in patients with biliary atresia: a preliminary study
title_fullStr Collagen gene cluster expression and liver fibrogenesis in patients with biliary atresia: a preliminary study
title_full_unstemmed Collagen gene cluster expression and liver fibrogenesis in patients with biliary atresia: a preliminary study
title_short Collagen gene cluster expression and liver fibrogenesis in patients with biliary atresia: a preliminary study
title_sort collagen gene cluster expression and liver fibrogenesis in patients with biliary atresia: a preliminary study
topic Research Note
url 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
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