Oligoclonal immunoglobulin repertoire in biliary remnants of biliary atresia
| Autor: | Sarah A. Taylor, Harris Perlman, Joshua B. Wechsler, Peter F. Whitington, Kathryn E. Hulse, Padmini Malladi, Xiaomin Pan |
|---|---|
| Rok vydání: | 2019 |
| Předmět: |
Pathology
medicine.medical_specialty medicine.medical_treatment lcsh:Medicine Immunoglobulins Liver transplantation Article 03 medical and health sciences Liver disease 0302 clinical medicine Immune system Antigen Biliary Atresia Biliary atresia medicine Humans lcsh:Science 030304 developmental biology B-Lymphocytes 0303 health sciences Multidisciplinary biology business.industry lcsh:R Infant Newborn High-Throughput Nucleotide Sequencing Infant Sequence Analysis DNA medicine.disease Phenotype 3. Good health Child Preschool 030220 oncology & carcinogenesis biology.protein lcsh:Q Lymph Antibody business |
| Zdroj: | Scientific Reports Scientific Reports, Vol 9, Iss 1, Pp 1-11 (2019) |
| ISSN: | 2045-2322 |
| DOI: | 10.1038/s41598-019-41148-7 |
| Popis: | Biliary atresia (BA) is a neonatal cholestatic liver disease that is the leading cause of pediatric liver transplantation, however, the mechanism of disease remains unknown. There are two major forms of BA: isolated BA (iBA) comprises the majority of cases and is thought to result from an aberrant immune response to an environmental trigger, whereas syndromic BA (BASM) has associated malformations and is thought to arise from a congenital insult. To determine whether B cells in BA biliary remnants are antigen driven, we examined the immunoglobulin (Ig) repertoire of diseased tissue from each BA group. Deep sequencing of the Ig chain DNA was performed on iBA and BASM biliary remnants and lymph nodes obtained from the Childhood Liver Disease Research Network (ChiLDReN) repository. Statistical analysis of the Ig repertoire provided measures of Ig clonality and the Ig phenotype. Our data demonstrate that B cells infiltrate diseased iBA and BASM biliary remnant tissue. The Ig repertoires of iBA and BASM disease groups were oligoclonal supporting a role for an antigen-driven immune response in both sub-types. These findings shift the current understanding of BA and suggest a role for antigen stimulation in early iBA and BASM disease pathogenesis. |
| Databáze: | OpenAIRE |
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