Collagen gene cluster expression and liver fibrogenesis in patients with biliary atresia: a preliminary study
Abstract 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...
| Main Authors: | , , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
BMC
2023-12-01
|
| Series: | BMC Research Notes |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s13104-023-06636-0 |
| _version_ | 1797416229010407424 |
|---|---|
| author | Gunadi Dyah Ayu Puspitarani Khanza Adzkia Vujira Fadila Dyah Trie Utami Edita Mayda Devana Fiqih Vidiantoro Halim Kristy Iskandar Akhmad Makhmudi |
| author_facet | Gunadi Dyah Ayu Puspitarani Khanza Adzkia Vujira Fadila Dyah Trie Utami Edita Mayda Devana Fiqih Vidiantoro Halim Kristy Iskandar Akhmad Makhmudi |
| author_sort | Gunadi |
| collection | DOAJ |
| description | Abstract 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 (ΔCT 9.06 ± 2.64 vs. 5.42 ± 2.41; p = 0.0009), COL6A2 (ΔCT 8.25 ± 2.07 vs. 5.77 ± 3.51; p = 0.02), COL6A3 (ΔCT 11.2 ± 6.08 vs. 6.78 ± 3.51; p = 0.024), and COL1A1 (ΔCT 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. |
| first_indexed | 2024-03-09T06:00:26Z |
| format | Article |
| id | doaj.art-ae7c92d31f25427888e06a3481c4cf1b |
| institution | Directory Open Access Journal |
| issn | 1756-0500 |
| language | English |
| last_indexed | 2024-03-09T06:00:26Z |
| publishDate | 2023-12-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Research Notes |
| spelling | doaj.art-ae7c92d31f25427888e06a3481c4cf1b2023-12-03T12:09:25ZengBMCBMC Research Notes1756-05002023-12-011611510.1186/s13104-023-06636-0Collagen gene cluster expression and liver fibrogenesis in patients with biliary atresia: a preliminary studyGunadi0Dyah Ayu Puspitarani1Khanza Adzkia Vujira2Fadila Dyah Trie Utami3Edita Mayda Devana4Fiqih Vidiantoro Halim5Kristy Iskandar6Akhmad Makhmudi7Pediatric Surgery Division, Department of Surgery/Genetics Working Group/Translational Research Unit, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada/Dr.Sardjito HospitalPediatric Surgery Division, Department of Surgery/Genetics Working Group/Translational Research Unit, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada/Dr.Sardjito HospitalPediatric Surgery Division, Department of Surgery/Genetics Working Group/Translational Research Unit, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada/Dr.Sardjito HospitalPediatric Surgery Division, Department of Surgery/Genetics Working Group/Translational Research Unit, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada/Dr.Sardjito HospitalPediatric Surgery Division, Department of Surgery/Genetics Working Group/Translational Research Unit, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada/Dr.Sardjito HospitalPediatric Surgery Division, Department of Surgery/Genetics Working Group/Translational Research Unit, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada/Dr.Sardjito HospitalDepartment of Child Health/Genetics Working Group, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada/UGM Academic HospitalPediatric Surgery Division, Department of Surgery/Genetics Working Group/Translational Research Unit, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada/Dr.Sardjito HospitalAbstract 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 (ΔCT 9.06 ± 2.64 vs. 5.42 ± 2.41; p = 0.0009), COL6A2 (ΔCT 8.25 ± 2.07 vs. 5.77 ± 3.51; p = 0.02), COL6A3 (ΔCT 11.2 ± 6.08 vs. 6.78 ± 3.51; p = 0.024), and COL1A1 (ΔCT 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.https://doi.org/10.1186/s13104-023-06636-0Biliary atresiaCollagen gene cluster expressionsDeveloping countryKasai procedureLiver fibrogenesis |
| spellingShingle | Gunadi Dyah Ayu Puspitarani Khanza Adzkia Vujira Fadila Dyah Trie Utami Edita Mayda Devana Fiqih Vidiantoro Halim Kristy Iskandar Akhmad Makhmudi Collagen gene cluster expression and liver fibrogenesis in patients with biliary atresia: a preliminary study BMC Research Notes Biliary atresia Collagen gene cluster expressions Developing country Kasai procedure Liver fibrogenesis |
| 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 | Biliary atresia Collagen gene cluster expressions Developing country Kasai procedure Liver fibrogenesis |
| url | https://doi.org/10.1186/s13104-023-06636-0 |
| work_keys_str_mv | AT gunadi collagengeneclusterexpressionandliverfibrogenesisinpatientswithbiliaryatresiaapreliminarystudy AT dyahayupuspitarani collagengeneclusterexpressionandliverfibrogenesisinpatientswithbiliaryatresiaapreliminarystudy AT khanzaadzkiavujira collagengeneclusterexpressionandliverfibrogenesisinpatientswithbiliaryatresiaapreliminarystudy AT fadiladyahtrieutami collagengeneclusterexpressionandliverfibrogenesisinpatientswithbiliaryatresiaapreliminarystudy AT editamaydadevana collagengeneclusterexpressionandliverfibrogenesisinpatientswithbiliaryatresiaapreliminarystudy AT fiqihvidiantorohalim collagengeneclusterexpressionandliverfibrogenesisinpatientswithbiliaryatresiaapreliminarystudy AT kristyiskandar collagengeneclusterexpressionandliverfibrogenesisinpatientswithbiliaryatresiaapreliminarystudy AT akhmadmakhmudi collagengeneclusterexpressionandliverfibrogenesisinpatientswithbiliaryatresiaapreliminarystudy |