Leucine-rich repeat kinase 2 promotes the development of experimental severe acute pancreatitis
Clin Exp Immunol. 2023 Oct 17:uxad106. doi: 10.1093/cei/uxad106. Online ahead of print.ABSTRACTTranslocation of gut bacteria into the pancreas promotes the development of severe acute pancreatitis (SAP). Recent clinical studies have also highlighted the association between fungal infections and SAP. The sensing of gut bacteria by pattern recognition receptors promotes the development of SAP via the production of proinflammatory cytokines; however, the mechanism by which gut fungi mediate SAP remains largely unknown. Leucine-rich repeat kinase 2 (LRRK2) is a multifunctional protein that regulates innate immunity against fun...
Source: Clinical and Developmental Immunology - October 17, 2023 Category: Allergy & Immunology Authors: Yasuo Otsuka Akane Hara Kosuke Minaga Ikue Sekai Masayuki Kurimoto Yasuhiro Masuta Ryutaro Takada Tomoe Yoshikawa Ken Kamata Masatoshi Kudo Tomohiro Watanabe Source Type: research

Leucine-rich repeat kinase 2 promotes the development of experimental severe acute pancreatitis
Clin Exp Immunol. 2023 Oct 17:uxad106. doi: 10.1093/cei/uxad106. Online ahead of print.ABSTRACTTranslocation of gut bacteria into the pancreas promotes the development of severe acute pancreatitis (SAP). Recent clinical studies have also highlighted the association between fungal infections and SAP. The sensing of gut bacteria by pattern recognition receptors promotes the development of SAP via the production of proinflammatory cytokines; however, the mechanism by which gut fungi mediate SAP remains largely unknown. Leucine-rich repeat kinase 2 (LRRK2) is a multifunctional protein that regulates innate immunity against fun...
Source: Clinical and Developmental Immunology - October 17, 2023 Category: Allergy & Immunology Authors: Yasuo Otsuka Akane Hara Kosuke Minaga Ikue Sekai Masayuki Kurimoto Yasuhiro Masuta Ryutaro Takada Tomoe Yoshikawa Ken Kamata Masatoshi Kudo Tomohiro Watanabe Source Type: research

Plasma levels of mannan-binding lectin-associated serine proteases are increased in type 1 diabetes patients with insulin resistance
In conclusion, IR in T1D amplifies levels of MASP-1/2/3 and their regulator MAp44, and improvement of IR normalises MASP-1/2/3 levels. Given that elevated levels of these proteins contribute to vascular pathology, amplification of the lectin pathway of the complement system may offer mechanistic insight into the relationship between IR and vascular complications in T1D.PMID:37832142 | DOI:10.1093/cei/uxad113 (Source: Clinical and Developmental Immunology)
Source: Clinical and Developmental Immunology - October 13, 2023 Category: Allergy & Immunology Authors: Noppadol Kietsiriroje Georgia E Scott Ramzi A Ajjan Jan Br ôz Verena Schroeder Matthew D Campbell Source Type: research

Plasma levels of mannan-binding lectin-associated serine proteases are increased in type 1 diabetes patients with insulin resistance
In conclusion, IR in T1D amplifies levels of MASP-1/2/3 and their regulator MAp44, and improvement of IR normalises MASP-1/2/3 levels. Given that elevated levels of these proteins contribute to vascular pathology, amplification of the lectin pathway of the complement system may offer mechanistic insight into the relationship between IR and vascular complications in T1D.PMID:37832142 | DOI:10.1093/cei/uxad113 (Source: Clinical and Developmental Immunology)
Source: Clinical and Developmental Immunology - October 13, 2023 Category: Allergy & Immunology Authors: Noppadol Kietsiriroje Georgia E Scott Ramzi A Ajjan Jan Br ôz Verena Schroeder Matthew D Campbell Source Type: research

Plasma levels of mannan-binding lectin-associated serine proteases are increased in type 1 diabetes patients with insulin resistance
In conclusion, IR in T1D amplifies levels of MASP-1/2/3 and their regulator MAp44, and improvement of IR normalises MASP-1/2/3 levels. Given that elevated levels of these proteins contribute to vascular pathology, amplification of the lectin pathway of the complement system may offer mechanistic insight into the relationship between IR and vascular complications in T1D.PMID:37832142 | DOI:10.1093/cei/uxad113 (Source: Clinical and Developmental Immunology)
Source: Clinical and Developmental Immunology - October 13, 2023 Category: Allergy & Immunology Authors: Noppadol Kietsiriroje Georgia E Scott Ramzi A Ajjan Jan Br ôz Verena Schroeder Matthew D Campbell Source Type: research

Plasma levels of mannan-binding lectin-associated serine proteases are increased in type 1 diabetes patients with insulin resistance
In conclusion, IR in T1D amplifies levels of MASP-1/2/3 and their regulator MAp44, and improvement of IR normalises MASP-1/2/3 levels. Given that elevated levels of these proteins contribute to vascular pathology, amplification of the lectin pathway of the complement system may offer mechanistic insight into the relationship between IR and vascular complications in T1D.PMID:37832142 | DOI:10.1093/cei/uxad113 (Source: Clinical and Developmental Immunology)
Source: Clinical and Developmental Immunology - October 13, 2023 Category: Allergy & Immunology Authors: Noppadol Kietsiriroje Georgia E Scott Ramzi A Ajjan Jan Br ôz Verena Schroeder Matthew D Campbell Source Type: research

Autoantibodies to beta tubulin in autoimmune liver diseases - relation to pANCA and clinical relevance
Clin Exp Immunol. 2023 Oct 12:uxad114. doi: 10.1093/cei/uxad114. Online ahead of print.ABSTRACTThere was evidence that pANCA (perinuclear antineutrophil cytoplasmic antibodies) in autoimmune liver diseases react with human beta-tubulin-5 (TBB5). Here we re-evaluate the specificity and clinical relevance of anti-TBB5 antibodies. Patients with untreated autoimmune hepatitis (AIH; n=53), AIH under immunosuppressive therapy (AIH-IS; n=125), primary sclerosing cholangitis (PSC; n=40), primary biliary cholangitis (PBC; n=250), non-autoimmune liver diseases (n=158), inflammatory bowel diseases (IBD; n=30), and healthy individuals...
Source: Clinical and Developmental Immunology - October 12, 2023 Category: Allergy & Immunology Authors: Beate Preu ß Amelie Frank Birgit Terjung Ulrich Spengler Christoph Berg Reinhild Klein Source Type: research

Autoantibodies to beta tubulin in autoimmune liver diseases - relation to pANCA and clinical relevance
Clin Exp Immunol. 2023 Oct 12:uxad114. doi: 10.1093/cei/uxad114. Online ahead of print.ABSTRACTThere was evidence that pANCA (perinuclear antineutrophil cytoplasmic antibodies) in autoimmune liver diseases react with human beta-tubulin-5 (TBB5). Here we re-evaluate the specificity and clinical relevance of anti-TBB5 antibodies. Patients with untreated autoimmune hepatitis (AIH; n=53), AIH under immunosuppressive therapy (AIH-IS; n=125), primary sclerosing cholangitis (PSC; n=40), primary biliary cholangitis (PBC; n=250), non-autoimmune liver diseases (n=158), inflammatory bowel diseases (IBD; n=30), and healthy individuals...
Source: Clinical and Developmental Immunology - October 12, 2023 Category: Allergy & Immunology Authors: Beate Preu ß Amelie Frank Birgit Terjung Ulrich Spengler Christoph Berg Reinhild Klein Source Type: research

Myelin oligodendrocyte glycoprotein antibody and N-methyl-d-aspartate receptor antibody overlapping syndrome: insights from the recent case reports
Clin Exp Immunol. 2023 Sep 19:uxad109. doi: 10.1093/cei/uxad109. Online ahead of print.ABSTRACTThe overlapping of two or more types of neural autoantibodies in one patient has increasingly been documented in recent years. The coexistence of myelin oligodendrocyte glycoprotein (MOG) and N-methyl-d-aspartate receptor (NMDAR) antibodies is most common, which leads to a unique condition known as the MOG antibody and NMDAR antibody overlapping syndrome (MNOS). Here, we have reviewed the pathogenesis, clinical manifestations, paraclinical features, and treatment of MNOS. Forty-nine patients with MNOS were included in this study....
Source: Clinical and Developmental Immunology - September 19, 2023 Category: Allergy & Immunology Authors: Bing-Qing Du Qi-Lun Lai Er-Chuang Li Meng-Ting Cai Gao-Li Fang Chun-Hong Shen Yin-Xi Zhang Mei-Ping Ding Source Type: research

Interleukin-11 and its eminent role in tissue fibrosis: A possible therapeutic target
Clin Exp Immunol. 2023 Sep 19:uxad108. doi: 10.1093/cei/uxad108. Online ahead of print.ABSTRACTInterleukin-11 is a cytokine from the IL-6 family of cytokines that includes IL-6 and OSM. Initially described for its role in platelet generation, it is now appreciated that this cytokine has multiple functions. Recently it has been found that IL-11 is critical in fibrosis in multiple different organ systems and systemically as in the autoimmune disease systemic sclerosis. Animal models of fibrosis have determined that animals with IL-11 receptor deletions have retarded fibrosis and that in wild type animals IL-11 is found at th...
Source: Clinical and Developmental Immunology - September 19, 2023 Category: Allergy & Immunology Authors: Steven O'Reilly Source Type: research

Heterogeneity in RAG 1/2 Deficiency: 35 Cases From A Single Center
This study aimed to define the clinical/laboratory spectrum of RAG1/2 deficiency. We retrospectively reviewed the clinical/laboratory data of 35 patients, grouped them as severe combined immunodeficiency (SCID), Omenn Syndrome (OS), and delayed-onset combined immunodeficiency (CID) and reported nine novel mutations. Male/female ratio was 23/12. Median age of clinical manifestations was 1 months (mo) (0.5-2), 2 mo (1.25-5), and 14 mo (3.63-27), age at diagnosis was 4 mo (3-6), 4.5 mo (2.5-9.75), and 27 mo (14.5-70) in SCID (n=25;71.4%), OS (n=5;14.3%), and CID (n=5;14.3%) patients, respectively. Common clinical manifestatio...
Source: Clinical and Developmental Immunology - September 19, 2023 Category: Allergy & Immunology Authors: Betul Karaatmaca Deniz Cagdas Saliha Esenboga Baran Erman Cagman Tan Tuba Turul Ozgur Kaan Boztug Mirjam Van Der Burg Ozden Sanal Ilhan Tezcan Source Type: research

Myelin oligodendrocyte glycoprotein antibody and N-methyl-d-aspartate receptor antibody overlapping syndrome: insights from the recent case reports
Clin Exp Immunol. 2023 Sep 19:uxad109. doi: 10.1093/cei/uxad109. Online ahead of print.ABSTRACTThe overlapping of two or more types of neural autoantibodies in one patient has increasingly been documented in recent years. The coexistence of myelin oligodendrocyte glycoprotein (MOG) and N-methyl-d-aspartate receptor (NMDAR) antibodies is most common, which leads to a unique condition known as the MOG antibody and NMDAR antibody overlapping syndrome (MNOS). Here, we have reviewed the pathogenesis, clinical manifestations, paraclinical features, and treatment of MNOS. Forty-nine patients with MNOS were included in this study....
Source: Clinical and Developmental Immunology - September 19, 2023 Category: Allergy & Immunology Authors: Bing-Qing Du Qi-Lun Lai Er-Chuang Li Meng-Ting Cai Gao-Li Fang Chun-Hong Shen Yin-Xi Zhang Mei-Ping Ding Source Type: research

Interleukin-11 and its eminent role in tissue fibrosis: A possible therapeutic target
Clin Exp Immunol. 2023 Sep 19:uxad108. doi: 10.1093/cei/uxad108. Online ahead of print.ABSTRACTInterleukin-11 is a cytokine from the IL-6 family of cytokines that includes IL-6 and OSM. Initially described for its role in platelet generation, it is now appreciated that this cytokine has multiple functions. Recently it has been found that IL-11 is critical in fibrosis in multiple different organ systems and systemically as in the autoimmune disease systemic sclerosis. Animal models of fibrosis have determined that animals with IL-11 receptor deletions have retarded fibrosis and that in wild type animals IL-11 is found at th...
Source: Clinical and Developmental Immunology - September 19, 2023 Category: Allergy & Immunology Authors: Steven O'Reilly Source Type: research

Heterogeneity in RAG 1/2 Deficiency: 35 Cases From A Single Center
This study aimed to define the clinical/laboratory spectrum of RAG1/2 deficiency. We retrospectively reviewed the clinical/laboratory data of 35 patients, grouped them as severe combined immunodeficiency (SCID), Omenn Syndrome (OS), and delayed-onset combined immunodeficiency (CID) and reported nine novel mutations. Male/female ratio was 23/12. Median age of clinical manifestations was 1 months (mo) (0.5-2), 2 mo (1.25-5), and 14 mo (3.63-27), age at diagnosis was 4 mo (3-6), 4.5 mo (2.5-9.75), and 27 mo (14.5-70) in SCID (n=25;71.4%), OS (n=5;14.3%), and CID (n=5;14.3%) patients, respectively. Common clinical manifestatio...
Source: Clinical and Developmental Immunology - September 19, 2023 Category: Allergy & Immunology Authors: Betul Karaatmaca Deniz Cagdas Saliha Esenboga Baran Erman Cagman Tan Tuba Turul Ozgur Kaan Boztug Mirjam Van Der Burg Ozden Sanal Ilhan Tezcan Source Type: research

Myelin oligodendrocyte glycoprotein antibody and N-methyl-d-aspartate receptor antibody overlapping syndrome: insights from the recent case reports
Clin Exp Immunol. 2023 Sep 19:uxad109. doi: 10.1093/cei/uxad109. Online ahead of print.ABSTRACTThe overlapping of two or more types of neural autoantibodies in one patient has increasingly been documented in recent years. The coexistence of myelin oligodendrocyte glycoprotein (MOG) and N-methyl-d-aspartate receptor (NMDAR) antibodies is most common, which leads to a unique condition known as the MOG antibody and NMDAR antibody overlapping syndrome (MNOS). Here, we have reviewed the pathogenesis, clinical manifestations, paraclinical features, and treatment of MNOS. Forty-nine patients with MNOS were included in this study....
Source: Clinical and Developmental Immunology - September 19, 2023 Category: Allergy & Immunology Authors: Bing-Qing Du Qi-Lun Lai Er-Chuang Li Meng-Ting Cai Gao-Li Fang Chun-Hong Shen Yin-Xi Zhang Mei-Ping Ding Source Type: research