중추관용

3. 중추관용의 기전

관용의 최종 결과는 자가 항원에 반응하지 않지만 무작위로 배열된 수용체에 따라 외래의 비자기 항원을 인식할 수 있는 림프구 집단이다.^[39] 림프구는 골수(B세포의 경우)와 흉선(T세포의 경우)에 존재하는 항원에 대해서만 관용을 획득할 수 있다.

3. 1. B 세포 관용

3. 2. T 세포 관용

4. 중추관용의 결함으로 인한 유전 질환

중추관용의 유전적 결함은 자가면역질환을 유발할 수 있다. 자가면역 다발성 내분비병증 증후군 제1형은 AIRE 유전자의 돌연변이로 인해 발생한다. 이로 인해 흉선에서 말초 항원의 발현이 부족해져 인슐린과 같은 주요 말초 단백질에 대한 음성 선택이 결핍되어, 여러 자가 면역 증상이 나타난다.^{[43][44][22][23][33][34]}

5. 중추관용의 역사

1945년, 레이 오웬(Ray Owen)은 이란성 쌍둥이 소에서 한쪽 소에게 다른 소의 혈액을 주입하여도 항체가 생성되지 않는 현상을 발견했다.^[45][24][35] 오웬의 발견은 이후 하세크(Hasek)와 빌링햄(Billingham)의 실험을 통해 확인되었다.^[45][24][35] 이 결과는 버넷의 클론 선택설로 설명되었다.^[46][25][36] 버넷과 메더워는 면역 관용 기전을 설명한 공로로 1960년 노벨 생리학·의학상을 수상했다.^{[46][47][25][26][36][37]}

6. 한국에서의 자가면역질환

참조

_[1] 서적 Kuby immunology W.H. Freeman 2013
_[2] 논문 Immunological tolerance and autoimmunity 2006
_[3] 서적 Immunobiology 5: The Immune System in Health and Disease https://archive.org/[...] Garland 2001
_[4] 논문 Autoimmunity due to RAG deficiency and estimated disease incidence in RAG1/2 mutations 2014-03
_[5] 서적 Immunobiology 5: The Immune System in Health and Disease Garland Science 2001
_[6] 논문 Central CD4⁺ T cell tolerance: deletion versus regulatory T cell differentiation https://epub.ub.uni-[...] 2019-01
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_[8] 논문 Tolerance is established in polyclonal CD4(+) T cells by distinct mechanisms, according to self-peptide expression patterns 2016-02
_[9] 논문 Formation of the Intrathymic Dendritic Cell Pool Requires CCL21-Mediated Recruitment of CCR7⁺ Progenitors to the Thymus 2018-07
_[10] 논문 Negative selection--clearing out the bad apples from the T-cell repertoire 2003-05
_[11] 논문 Regulatory T cells prevent catastrophic autoimmunity throughout the lifespan of mice 2007-02
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_[15] 논문 CCR7 and CCR9 together recruit hematopoietic progenitors to the adult thymus https://ashpublicati[...] 2010-03-11
_[16] 논문 Hematopoietic progenitor migration to the adult thymus 2011
_[17] 논문 An Overview of the Intrathymic Intricacies of T Cell Development https://journals.aai[...] 2014-05-01
_[18] 논문 Single-cell insights into the hematopoietic generation of T-lymphocyte precursors in mouse and human 2021
_[19] 논문 Regulation of γδ T Cell Effector Diversification in the Thymus 2020
_[20] 논문 Determining γδ versus αß T cell development https://pubmed.ncbi.[...] 2010
_[21] 논문 Reciprocal developmental pathways for the generation of pathogenic effector TH17 and regulatory T cells 2006-05
_[22] 논문 Projection of an immunological self shadow within the thymus by the aire protein 2002-11
_[23] 논문 Aire regulates negative selection of organ-specific T cells 2003-04
_[24] 논문 Historical overview of immunological tolerance 2012-04
_[25] 논문 Immunological tolerance 50 years after the Burnet Nobel Prize 2011-01
_[26] 논문 The curious case of the 1960 Nobel Prize to Burnet and Medawar 2016-03
_[27] 서적 Kuby immunology W.H. Freeman 2013
_[28] 논문 Immunological tolerance and autoimmunity 2006
_[29] 서적 Immunobiology 5: The Immune System in Health and Disease https://archive.org/[...] Garland 2011
_[30] 논문 Autoimmunity due to RAG deficiency and estimated disease incidence in RAG1/2 mutations 2014-03
_[31] 논문 The thymus and central tolerance 2001-05
_[32] 논문 Reciprocal developmental pathways for the generation of pathogenic effector TH17 and regulatory T cells 2006-05
_[33] 논문 Projection of an immunological self shadow within the thymus by the aire protein 2002-11
_[34] 논문 Aire regulates negative selection of organ-specific T cells 2003-04
_[35] 논문 Historical overview of immunological tolerance 2012-04
_[36] 논문 Immunological tolerance 50 years after the Burnet Nobel Prize 2011-01
_[37] 논문 The curious case of the 1960 Nobel Prize to Burnet and Medawar 2016-03
_[38] 서적 Kuby immunology https://archive.org/[...] W.H. Freeman 2013
_[39] 저널 Immunological tolerance and autoimmunity 2006
_[40] 서적 Immunobiology 5: The Immune System in Health and Disease https://archive.org/[...] Garland 2011
_[41] 저널 Autoimmunity due to RAG deficiency and estimated disease incidence in RAG1/2 mutations 2014-03
_[42] 저널 Reciprocal developmental pathways for the generation of pathogenic effector TH17 and regulatory T cells 2006-05
_[43] 저널 Projection of an immunological self shadow within the thymus by the aire protein 2002-11
_[44] 저널 Aire regulates negative selection of organ-specific T cells 2003-04
_[45] 저널 Historical overview of immunological tolerance 2012-04
_[46] 저널 Immunological tolerance 50 years after the Burnet Nobel Prize 2011-01
_[47] 저널 The curious case of the 1960 Nobel Prize to Burnet and Medawar 2016-03