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ResearchIn-Press PreviewGeneticsNephrology
Open Access | 10.1172/JCI181164
1Division of Nephrology, Columbia University, New York, United States of America
2Department of Immunology, University of Alabama at Birmingham, Birmingham, Czech Republic
3Department of Immunology, University of Alabama at Birmingham, Birmingham, United States of America
4Division of Infectious Diseases, Columbia University, New York, United States of America
5Departments of Medicine and Surgery, University of Parma, Parma, Italy
6Division of Nephrology, Columbia University, New York, Italy
7Department of Cell Biology and Molecular Medicine, Rutgers Biomedical and Health Sciences, Newark, United States of America
8Division of Nephrology, University of Alabama at Birmingham, Birmingham, United States of America
9The Renal Division, Peking University First Hospital, Beijing, China
10Division of Renal Pathology, Columbia University, New York, United States of America
11Department of Microbiology, University of Alabama at Birmingham, Birmingham, United States of America
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1Division of Nephrology, Columbia University, New York, United States of America
2Department of Immunology, University of Alabama at Birmingham, Birmingham, Czech Republic
3Department of Immunology, University of Alabama at Birmingham, Birmingham, United States of America
4Division of Infectious Diseases, Columbia University, New York, United States of America
5Departments of Medicine and Surgery, University of Parma, Parma, Italy
6Division of Nephrology, Columbia University, New York, Italy
7Department of Cell Biology and Molecular Medicine, Rutgers Biomedical and Health Sciences, Newark, United States of America
8Division of Nephrology, University of Alabama at Birmingham, Birmingham, United States of America
9The Renal Division, Peking University First Hospital, Beijing, China
10Division of Renal Pathology, Columbia University, New York, United States of America
11Department of Microbiology, University of Alabama at Birmingham, Birmingham, United States of America
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1Division of Nephrology, Columbia University, New York, United States of America
2Department of Immunology, University of Alabama at Birmingham, Birmingham, Czech Republic
3Department of Immunology, University of Alabama at Birmingham, Birmingham, United States of America
4Division of Infectious Diseases, Columbia University, New York, United States of America
5Departments of Medicine and Surgery, University of Parma, Parma, Italy
6Division of Nephrology, Columbia University, New York, Italy
7Department of Cell Biology and Molecular Medicine, Rutgers Biomedical and Health Sciences, Newark, United States of America
8Division of Nephrology, University of Alabama at Birmingham, Birmingham, United States of America
9The Renal Division, Peking University First Hospital, Beijing, China
10Division of Renal Pathology, Columbia University, New York, United States of America
11Department of Microbiology, University of Alabama at Birmingham, Birmingham, United States of America
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1Division of Nephrology, Columbia University, New York, United States of America
2Department of Immunology, University of Alabama at Birmingham, Birmingham, Czech Republic
3Department of Immunology, University of Alabama at Birmingham, Birmingham, United States of America
4Division of Infectious Diseases, Columbia University, New York, United States of America
5Departments of Medicine and Surgery, University of Parma, Parma, Italy
6Division of Nephrology, Columbia University, New York, Italy
7Department of Cell Biology and Molecular Medicine, Rutgers Biomedical and Health Sciences, Newark, United States of America
8Division of Nephrology, University of Alabama at Birmingham, Birmingham, United States of America
9The Renal Division, Peking University First Hospital, Beijing, China
10Division of Renal Pathology, Columbia University, New York, United States of America
11Department of Microbiology, University of Alabama at Birmingham, Birmingham, United States of America
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1Division of Nephrology, Columbia University, New York, United States of America
2Department of Immunology, University of Alabama at Birmingham, Birmingham, Czech Republic
3Department of Immunology, University of Alabama at Birmingham, Birmingham, United States of America
4Division of Infectious Diseases, Columbia University, New York, United States of America
5Departments of Medicine and Surgery, University of Parma, Parma, Italy
6Division of Nephrology, Columbia University, New York, Italy
7Department of Cell Biology and Molecular Medicine, Rutgers Biomedical and Health Sciences, Newark, United States of America
8Division of Nephrology, University of Alabama at Birmingham, Birmingham, United States of America
9The Renal Division, Peking University First Hospital, Beijing, China
10Division of Renal Pathology, Columbia University, New York, United States of America
11Department of Microbiology, University of Alabama at Birmingham, Birmingham, United States of America
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1Division of Nephrology, Columbia University, New York, United States of America
2Department of Immunology, University of Alabama at Birmingham, Birmingham, Czech Republic
3Department of Immunology, University of Alabama at Birmingham, Birmingham, United States of America
4Division of Infectious Diseases, Columbia University, New York, United States of America
5Departments of Medicine and Surgery, University of Parma, Parma, Italy
6Division of Nephrology, Columbia University, New York, Italy
7Department of Cell Biology and Molecular Medicine, Rutgers Biomedical and Health Sciences, Newark, United States of America
8Division of Nephrology, University of Alabama at Birmingham, Birmingham, United States of America
9The Renal Division, Peking University First Hospital, Beijing, China
10Division of Renal Pathology, Columbia University, New York, United States of America
11Department of Microbiology, University of Alabama at Birmingham, Birmingham, United States of America
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1Division of Nephrology, Columbia University, New York, United States of America
2Department of Immunology, University of Alabama at Birmingham, Birmingham, Czech Republic
3Department of Immunology, University of Alabama at Birmingham, Birmingham, United States of America
4Division of Infectious Diseases, Columbia University, New York, United States of America
5Departments of Medicine and Surgery, University of Parma, Parma, Italy
6Division of Nephrology, Columbia University, New York, Italy
7Department of Cell Biology and Molecular Medicine, Rutgers Biomedical and Health Sciences, Newark, United States of America
8Division of Nephrology, University of Alabama at Birmingham, Birmingham, United States of America
9The Renal Division, Peking University First Hospital, Beijing, China
10Division of Renal Pathology, Columbia University, New York, United States of America
11Department of Microbiology, University of Alabama at Birmingham, Birmingham, United States of America
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1Division of Nephrology, Columbia University, New York, United States of America
2Department of Immunology, University of Alabama at Birmingham, Birmingham, Czech Republic
3Department of Immunology, University of Alabama at Birmingham, Birmingham, United States of America
4Division of Infectious Diseases, Columbia University, New York, United States of America
5Departments of Medicine and Surgery, University of Parma, Parma, Italy
6Division of Nephrology, Columbia University, New York, Italy
7Department of Cell Biology and Molecular Medicine, Rutgers Biomedical and Health Sciences, Newark, United States of America
8Division of Nephrology, University of Alabama at Birmingham, Birmingham, United States of America
9The Renal Division, Peking University First Hospital, Beijing, China
10Division of Renal Pathology, Columbia University, New York, United States of America
11Department of Microbiology, University of Alabama at Birmingham, Birmingham, United States of America
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1Division of Nephrology, Columbia University, New York, United States of America
2Department of Immunology, University of Alabama at Birmingham, Birmingham, Czech Republic
3Department of Immunology, University of Alabama at Birmingham, Birmingham, United States of America
4Division of Infectious Diseases, Columbia University, New York, United States of America
5Departments of Medicine and Surgery, University of Parma, Parma, Italy
6Division of Nephrology, Columbia University, New York, Italy
7Department of Cell Biology and Molecular Medicine, Rutgers Biomedical and Health Sciences, Newark, United States of America
8Division of Nephrology, University of Alabama at Birmingham, Birmingham, United States of America
9The Renal Division, Peking University First Hospital, Beijing, China
10Division of Renal Pathology, Columbia University, New York, United States of America
11Department of Microbiology, University of Alabama at Birmingham, Birmingham, United States of America
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1Division of Nephrology, Columbia University, New York, United States of America
2Department of Immunology, University of Alabama at Birmingham, Birmingham, Czech Republic
3Department of Immunology, University of Alabama at Birmingham, Birmingham, United States of America
4Division of Infectious Diseases, Columbia University, New York, United States of America
5Departments of Medicine and Surgery, University of Parma, Parma, Italy
6Division of Nephrology, Columbia University, New York, Italy
7Department of Cell Biology and Molecular Medicine, Rutgers Biomedical and Health Sciences, Newark, United States of America
8Division of Nephrology, University of Alabama at Birmingham, Birmingham, United States of America
9The Renal Division, Peking University First Hospital, Beijing, China
10Division of Renal Pathology, Columbia University, New York, United States of America
11Department of Microbiology, University of Alabama at Birmingham, Birmingham, United States of America
Find articles by Reily, C. in: JCI | PubMed | Google Scholar
1Division of Nephrology, Columbia University, New York, United States of America
2Department of Immunology, University of Alabama at Birmingham, Birmingham, Czech Republic
3Department of Immunology, University of Alabama at Birmingham, Birmingham, United States of America
4Division of Infectious Diseases, Columbia University, New York, United States of America
5Departments of Medicine and Surgery, University of Parma, Parma, Italy
6Division of Nephrology, Columbia University, New York, Italy
7Department of Cell Biology and Molecular Medicine, Rutgers Biomedical and Health Sciences, Newark, United States of America
8Division of Nephrology, University of Alabama at Birmingham, Birmingham, United States of America
9The Renal Division, Peking University First Hospital, Beijing, China
10Division of Renal Pathology, Columbia University, New York, United States of America
11Department of Microbiology, University of Alabama at Birmingham, Birmingham, United States of America
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1Division of Nephrology, Columbia University, New York, United States of America
2Department of Immunology, University of Alabama at Birmingham, Birmingham, Czech Republic
3Department of Immunology, University of Alabama at Birmingham, Birmingham, United States of America
4Division of Infectious Diseases, Columbia University, New York, United States of America
5Departments of Medicine and Surgery, University of Parma, Parma, Italy
6Division of Nephrology, Columbia University, New York, Italy
7Department of Cell Biology and Molecular Medicine, Rutgers Biomedical and Health Sciences, Newark, United States of America
8Division of Nephrology, University of Alabama at Birmingham, Birmingham, United States of America
9The Renal Division, Peking University First Hospital, Beijing, China
10Division of Renal Pathology, Columbia University, New York, United States of America
11Department of Microbiology, University of Alabama at Birmingham, Birmingham, United States of America
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1Division of Nephrology, Columbia University, New York, United States of America
2Department of Immunology, University of Alabama at Birmingham, Birmingham, Czech Republic
3Department of Immunology, University of Alabama at Birmingham, Birmingham, United States of America
4Division of Infectious Diseases, Columbia University, New York, United States of America
5Departments of Medicine and Surgery, University of Parma, Parma, Italy
6Division of Nephrology, Columbia University, New York, Italy
7Department of Cell Biology and Molecular Medicine, Rutgers Biomedical and Health Sciences, Newark, United States of America
8Division of Nephrology, University of Alabama at Birmingham, Birmingham, United States of America
9The Renal Division, Peking University First Hospital, Beijing, China
10Division of Renal Pathology, Columbia University, New York, United States of America
11Department of Microbiology, University of Alabama at Birmingham, Birmingham, United States of America
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1Division of Nephrology, Columbia University, New York, United States of America
2Department of Immunology, University of Alabama at Birmingham, Birmingham, Czech Republic
3Department of Immunology, University of Alabama at Birmingham, Birmingham, United States of America
4Division of Infectious Diseases, Columbia University, New York, United States of America
5Departments of Medicine and Surgery, University of Parma, Parma, Italy
6Division of Nephrology, Columbia University, New York, Italy
7Department of Cell Biology and Molecular Medicine, Rutgers Biomedical and Health Sciences, Newark, United States of America
8Division of Nephrology, University of Alabama at Birmingham, Birmingham, United States of America
9The Renal Division, Peking University First Hospital, Beijing, China
10Division of Renal Pathology, Columbia University, New York, United States of America
11Department of Microbiology, University of Alabama at Birmingham, Birmingham, United States of America
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1Division of Nephrology, Columbia University, New York, United States of America
2Department of Immunology, University of Alabama at Birmingham, Birmingham, Czech Republic
3Department of Immunology, University of Alabama at Birmingham, Birmingham, United States of America
4Division of Infectious Diseases, Columbia University, New York, United States of America
5Departments of Medicine and Surgery, University of Parma, Parma, Italy
6Division of Nephrology, Columbia University, New York, Italy
7Department of Cell Biology and Molecular Medicine, Rutgers Biomedical and Health Sciences, Newark, United States of America
8Division of Nephrology, University of Alabama at Birmingham, Birmingham, United States of America
9The Renal Division, Peking University First Hospital, Beijing, China
10Division of Renal Pathology, Columbia University, New York, United States of America
11Department of Microbiology, University of Alabama at Birmingham, Birmingham, United States of America
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1Division of Nephrology, Columbia University, New York, United States of America
2Department of Immunology, University of Alabama at Birmingham, Birmingham, Czech Republic
3Department of Immunology, University of Alabama at Birmingham, Birmingham, United States of America
4Division of Infectious Diseases, Columbia University, New York, United States of America
5Departments of Medicine and Surgery, University of Parma, Parma, Italy
6Division of Nephrology, Columbia University, New York, Italy
7Department of Cell Biology and Molecular Medicine, Rutgers Biomedical and Health Sciences, Newark, United States of America
8Division of Nephrology, University of Alabama at Birmingham, Birmingham, United States of America
9The Renal Division, Peking University First Hospital, Beijing, China
10Division of Renal Pathology, Columbia University, New York, United States of America
11Department of Microbiology, University of Alabama at Birmingham, Birmingham, United States of America
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1Division of Nephrology, Columbia University, New York, United States of America
2Department of Immunology, University of Alabama at Birmingham, Birmingham, Czech Republic
3Department of Immunology, University of Alabama at Birmingham, Birmingham, United States of America
4Division of Infectious Diseases, Columbia University, New York, United States of America
5Departments of Medicine and Surgery, University of Parma, Parma, Italy
6Division of Nephrology, Columbia University, New York, Italy
7Department of Cell Biology and Molecular Medicine, Rutgers Biomedical and Health Sciences, Newark, United States of America
8Division of Nephrology, University of Alabama at Birmingham, Birmingham, United States of America
9The Renal Division, Peking University First Hospital, Beijing, China
10Division of Renal Pathology, Columbia University, New York, United States of America
11Department of Microbiology, University of Alabama at Birmingham, Birmingham, United States of America
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1Division of Nephrology, Columbia University, New York, United States of America
2Department of Immunology, University of Alabama at Birmingham, Birmingham, Czech Republic
3Department of Immunology, University of Alabama at Birmingham, Birmingham, United States of America
4Division of Infectious Diseases, Columbia University, New York, United States of America
5Departments of Medicine and Surgery, University of Parma, Parma, Italy
6Division of Nephrology, Columbia University, New York, Italy
7Department of Cell Biology and Molecular Medicine, Rutgers Biomedical and Health Sciences, Newark, United States of America
8Division of Nephrology, University of Alabama at Birmingham, Birmingham, United States of America
9The Renal Division, Peking University First Hospital, Beijing, China
10Division of Renal Pathology, Columbia University, New York, United States of America
11Department of Microbiology, University of Alabama at Birmingham, Birmingham, United States of America
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1Division of Nephrology, Columbia University, New York, United States of America
2Department of Immunology, University of Alabama at Birmingham, Birmingham, Czech Republic
3Department of Immunology, University of Alabama at Birmingham, Birmingham, United States of America
4Division of Infectious Diseases, Columbia University, New York, United States of America
5Departments of Medicine and Surgery, University of Parma, Parma, Italy
6Division of Nephrology, Columbia University, New York, Italy
7Department of Cell Biology and Molecular Medicine, Rutgers Biomedical and Health Sciences, Newark, United States of America
8Division of Nephrology, University of Alabama at Birmingham, Birmingham, United States of America
9The Renal Division, Peking University First Hospital, Beijing, China
10Division of Renal Pathology, Columbia University, New York, United States of America
11Department of Microbiology, University of Alabama at Birmingham, Birmingham, United States of America
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1Division of Nephrology, Columbia University, New York, United States of America
2Department of Immunology, University of Alabama at Birmingham, Birmingham, Czech Republic
3Department of Immunology, University of Alabama at Birmingham, Birmingham, United States of America
4Division of Infectious Diseases, Columbia University, New York, United States of America
5Departments of Medicine and Surgery, University of Parma, Parma, Italy
6Division of Nephrology, Columbia University, New York, Italy
7Department of Cell Biology and Molecular Medicine, Rutgers Biomedical and Health Sciences, Newark, United States of America
8Division of Nephrology, University of Alabama at Birmingham, Birmingham, United States of America
9The Renal Division, Peking University First Hospital, Beijing, China
10Division of Renal Pathology, Columbia University, New York, United States of America
11Department of Microbiology, University of Alabama at Birmingham, Birmingham, United States of America
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1Division of Nephrology, Columbia University, New York, United States of America
2Department of Immunology, University of Alabama at Birmingham, Birmingham, Czech Republic
3Department of Immunology, University of Alabama at Birmingham, Birmingham, United States of America
4Division of Infectious Diseases, Columbia University, New York, United States of America
5Departments of Medicine and Surgery, University of Parma, Parma, Italy
6Division of Nephrology, Columbia University, New York, Italy
7Department of Cell Biology and Molecular Medicine, Rutgers Biomedical and Health Sciences, Newark, United States of America
8Division of Nephrology, University of Alabama at Birmingham, Birmingham, United States of America
9The Renal Division, Peking University First Hospital, Beijing, China
10Division of Renal Pathology, Columbia University, New York, United States of America
11Department of Microbiology, University of Alabama at Birmingham, Birmingham, United States of America
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1Division of Nephrology, Columbia University, New York, United States of America
2Department of Immunology, University of Alabama at Birmingham, Birmingham, Czech Republic
3Department of Immunology, University of Alabama at Birmingham, Birmingham, United States of America
4Division of Infectious Diseases, Columbia University, New York, United States of America
5Departments of Medicine and Surgery, University of Parma, Parma, Italy
6Division of Nephrology, Columbia University, New York, Italy
7Department of Cell Biology and Molecular Medicine, Rutgers Biomedical and Health Sciences, Newark, United States of America
8Division of Nephrology, University of Alabama at Birmingham, Birmingham, United States of America
9The Renal Division, Peking University First Hospital, Beijing, China
10Division of Renal Pathology, Columbia University, New York, United States of America
11Department of Microbiology, University of Alabama at Birmingham, Birmingham, United States of America
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1Division of Nephrology, Columbia University, New York, United States of America
2Department of Immunology, University of Alabama at Birmingham, Birmingham, Czech Republic
3Department of Immunology, University of Alabama at Birmingham, Birmingham, United States of America
4Division of Infectious Diseases, Columbia University, New York, United States of America
5Departments of Medicine and Surgery, University of Parma, Parma, Italy
6Division of Nephrology, Columbia University, New York, Italy
7Department of Cell Biology and Molecular Medicine, Rutgers Biomedical and Health Sciences, Newark, United States of America
8Division of Nephrology, University of Alabama at Birmingham, Birmingham, United States of America
9The Renal Division, Peking University First Hospital, Beijing, China
10Division of Renal Pathology, Columbia University, New York, United States of America
11Department of Microbiology, University of Alabama at Birmingham, Birmingham, United States of America
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1Division of Nephrology, Columbia University, New York, United States of America
2Department of Immunology, University of Alabama at Birmingham, Birmingham, Czech Republic
3Department of Immunology, University of Alabama at Birmingham, Birmingham, United States of America
4Division of Infectious Diseases, Columbia University, New York, United States of America
5Departments of Medicine and Surgery, University of Parma, Parma, Italy
6Division of Nephrology, Columbia University, New York, Italy
7Department of Cell Biology and Molecular Medicine, Rutgers Biomedical and Health Sciences, Newark, United States of America
8Division of Nephrology, University of Alabama at Birmingham, Birmingham, United States of America
9The Renal Division, Peking University First Hospital, Beijing, China
10Division of Renal Pathology, Columbia University, New York, United States of America
11Department of Microbiology, University of Alabama at Birmingham, Birmingham, United States of America
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1Division of Nephrology, Columbia University, New York, United States of America
2Department of Immunology, University of Alabama at Birmingham, Birmingham, Czech Republic
3Department of Immunology, University of Alabama at Birmingham, Birmingham, United States of America
4Division of Infectious Diseases, Columbia University, New York, United States of America
5Departments of Medicine and Surgery, University of Parma, Parma, Italy
6Division of Nephrology, Columbia University, New York, Italy
7Department of Cell Biology and Molecular Medicine, Rutgers Biomedical and Health Sciences, Newark, United States of America
8Division of Nephrology, University of Alabama at Birmingham, Birmingham, United States of America
9The Renal Division, Peking University First Hospital, Beijing, China
10Division of Renal Pathology, Columbia University, New York, United States of America
11Department of Microbiology, University of Alabama at Birmingham, Birmingham, United States of America
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1Division of Nephrology, Columbia University, New York, United States of America
2Department of Immunology, University of Alabama at Birmingham, Birmingham, Czech Republic
3Department of Immunology, University of Alabama at Birmingham, Birmingham, United States of America
4Division of Infectious Diseases, Columbia University, New York, United States of America
5Departments of Medicine and Surgery, University of Parma, Parma, Italy
6Division of Nephrology, Columbia University, New York, Italy
7Department of Cell Biology and Molecular Medicine, Rutgers Biomedical and Health Sciences, Newark, United States of America
8Division of Nephrology, University of Alabama at Birmingham, Birmingham, United States of America
9The Renal Division, Peking University First Hospital, Beijing, China
10Division of Renal Pathology, Columbia University, New York, United States of America
11Department of Microbiology, University of Alabama at Birmingham, Birmingham, United States of America
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1Division of Nephrology, Columbia University, New York, United States of America
2Department of Immunology, University of Alabama at Birmingham, Birmingham, Czech Republic
3Department of Immunology, University of Alabama at Birmingham, Birmingham, United States of America
4Division of Infectious Diseases, Columbia University, New York, United States of America
5Departments of Medicine and Surgery, University of Parma, Parma, Italy
6Division of Nephrology, Columbia University, New York, Italy
7Department of Cell Biology and Molecular Medicine, Rutgers Biomedical and Health Sciences, Newark, United States of America
8Division of Nephrology, University of Alabama at Birmingham, Birmingham, United States of America
9The Renal Division, Peking University First Hospital, Beijing, China
10Division of Renal Pathology, Columbia University, New York, United States of America
11Department of Microbiology, University of Alabama at Birmingham, Birmingham, United States of America
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1Division of Nephrology, Columbia University, New York, United States of America
2Department of Immunology, University of Alabama at Birmingham, Birmingham, Czech Republic
3Department of Immunology, University of Alabama at Birmingham, Birmingham, United States of America
4Division of Infectious Diseases, Columbia University, New York, United States of America
5Departments of Medicine and Surgery, University of Parma, Parma, Italy
6Division of Nephrology, Columbia University, New York, Italy
7Department of Cell Biology and Molecular Medicine, Rutgers Biomedical and Health Sciences, Newark, United States of America
8Division of Nephrology, University of Alabama at Birmingham, Birmingham, United States of America
9The Renal Division, Peking University First Hospital, Beijing, China
10Division of Renal Pathology, Columbia University, New York, United States of America
11Department of Microbiology, University of Alabama at Birmingham, Birmingham, United States of America
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1Division of Nephrology, Columbia University, New York, United States of America
2Department of Immunology, University of Alabama at Birmingham, Birmingham, Czech Republic
3Department of Immunology, University of Alabama at Birmingham, Birmingham, United States of America
4Division of Infectious Diseases, Columbia University, New York, United States of America
5Departments of Medicine and Surgery, University of Parma, Parma, Italy
6Division of Nephrology, Columbia University, New York, Italy
7Department of Cell Biology and Molecular Medicine, Rutgers Biomedical and Health Sciences, Newark, United States of America
8Division of Nephrology, University of Alabama at Birmingham, Birmingham, United States of America
9The Renal Division, Peking University First Hospital, Beijing, China
10Division of Renal Pathology, Columbia University, New York, United States of America
11Department of Microbiology, University of Alabama at Birmingham, Birmingham, United States of America
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1Division of Nephrology, Columbia University, New York, United States of America
2Department of Immunology, University of Alabama at Birmingham, Birmingham, Czech Republic
3Department of Immunology, University of Alabama at Birmingham, Birmingham, United States of America
4Division of Infectious Diseases, Columbia University, New York, United States of America
5Departments of Medicine and Surgery, University of Parma, Parma, Italy
6Division of Nephrology, Columbia University, New York, Italy
7Department of Cell Biology and Molecular Medicine, Rutgers Biomedical and Health Sciences, Newark, United States of America
8Division of Nephrology, University of Alabama at Birmingham, Birmingham, United States of America
9The Renal Division, Peking University First Hospital, Beijing, China
10Division of Renal Pathology, Columbia University, New York, United States of America
11Department of Microbiology, University of Alabama at Birmingham, Birmingham, United States of America
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1Division of Nephrology, Columbia University, New York, United States of America
2Department of Immunology, University of Alabama at Birmingham, Birmingham, Czech Republic
3Department of Immunology, University of Alabama at Birmingham, Birmingham, United States of America
4Division of Infectious Diseases, Columbia University, New York, United States of America
5Departments of Medicine and Surgery, University of Parma, Parma, Italy
6Division of Nephrology, Columbia University, New York, Italy
7Department of Cell Biology and Molecular Medicine, Rutgers Biomedical and Health Sciences, Newark, United States of America
8Division of Nephrology, University of Alabama at Birmingham, Birmingham, United States of America
9The Renal Division, Peking University First Hospital, Beijing, China
10Division of Renal Pathology, Columbia University, New York, United States of America
11Department of Microbiology, University of Alabama at Birmingham, Birmingham, United States of America
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1Division of Nephrology, Columbia University, New York, United States of America
2Department of Immunology, University of Alabama at Birmingham, Birmingham, Czech Republic
3Department of Immunology, University of Alabama at Birmingham, Birmingham, United States of America
4Division of Infectious Diseases, Columbia University, New York, United States of America
5Departments of Medicine and Surgery, University of Parma, Parma, Italy
6Division of Nephrology, Columbia University, New York, Italy
7Department of Cell Biology and Molecular Medicine, Rutgers Biomedical and Health Sciences, Newark, United States of America
8Division of Nephrology, University of Alabama at Birmingham, Birmingham, United States of America
9The Renal Division, Peking University First Hospital, Beijing, China
10Division of Renal Pathology, Columbia University, New York, United States of America
11Department of Microbiology, University of Alabama at Birmingham, Birmingham, United States of America
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1Division of Nephrology, Columbia University, New York, United States of America
2Department of Immunology, University of Alabama at Birmingham, Birmingham, Czech Republic
3Department of Immunology, University of Alabama at Birmingham, Birmingham, United States of America
4Division of Infectious Diseases, Columbia University, New York, United States of America
5Departments of Medicine and Surgery, University of Parma, Parma, Italy
6Division of Nephrology, Columbia University, New York, Italy
7Department of Cell Biology and Molecular Medicine, Rutgers Biomedical and Health Sciences, Newark, United States of America
8Division of Nephrology, University of Alabama at Birmingham, Birmingham, United States of America
9The Renal Division, Peking University First Hospital, Beijing, China
10Division of Renal Pathology, Columbia University, New York, United States of America
11Department of Microbiology, University of Alabama at Birmingham, Birmingham, United States of America
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1Division of Nephrology, Columbia University, New York, United States of America
2Department of Immunology, University of Alabama at Birmingham, Birmingham, Czech Republic
3Department of Immunology, University of Alabama at Birmingham, Birmingham, United States of America
4Division of Infectious Diseases, Columbia University, New York, United States of America
5Departments of Medicine and Surgery, University of Parma, Parma, Italy
6Division of Nephrology, Columbia University, New York, Italy
7Department of Cell Biology and Molecular Medicine, Rutgers Biomedical and Health Sciences, Newark, United States of America
8Division of Nephrology, University of Alabama at Birmingham, Birmingham, United States of America
9The Renal Division, Peking University First Hospital, Beijing, China
10Division of Renal Pathology, Columbia University, New York, United States of America
11Department of Microbiology, University of Alabama at Birmingham, Birmingham, United States of America
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1Division of Nephrology, Columbia University, New York, United States of America
2Department of Immunology, University of Alabama at Birmingham, Birmingham, Czech Republic
3Department of Immunology, University of Alabama at Birmingham, Birmingham, United States of America
4Division of Infectious Diseases, Columbia University, New York, United States of America
5Departments of Medicine and Surgery, University of Parma, Parma, Italy
6Division of Nephrology, Columbia University, New York, Italy
7Department of Cell Biology and Molecular Medicine, Rutgers Biomedical and Health Sciences, Newark, United States of America
8Division of Nephrology, University of Alabama at Birmingham, Birmingham, United States of America
9The Renal Division, Peking University First Hospital, Beijing, China
10Division of Renal Pathology, Columbia University, New York, United States of America
11Department of Microbiology, University of Alabama at Birmingham, Birmingham, United States of America
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Gharavi, A.
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Published March 28, 2025 - More info
Aberrant O-glycosylation of the IgA1 hinge region is a characteristic finding in patients with IgA nephropathy (IgAN) and is thought to contribute to immune-complex formation and kidney injury. Other studies have suggested that abnormalities in mucosal immunity and lymphocyte homing are major contributors to disease. We identified a family with IgAN segregating a heterozygous predicted loss-of-function (LOF) variant in GALNT14, the gene encoding N-acetylgalactosaminyltransferase 14, one of the enzymes involved in mucin-type protein O-glycosylation. While GALNT14 is expressed in IgA1-producing cells, carriers of the LOF variant did not have altered levels of poorly glycosylated IgA1, suggesting other disease mechanisms. Investigation of Galnt14 null mice revealed elevated serum IgA levels and ex vivo IgA production by B cells. These mice developed glomerular IgA deposition with aging and after induction of sterile colitis. Galnt14 null mice also displayed an attenuated mucin layer in the colon and redistribution of IgA-producing cells from mucosal to systemic sites. Adoptive-transfer experiments indicated impaired homing of spleen-derived Galnt14 deficient B lymphocytes, resulting in increased retention in peripheral blood. These findings suggest that abnormalities in O-glycosylation alter mucosal immunity and B lymphocyte homing, pointing to an expanded role of aberrant O-glycosylation in the pathogenesis of IgAN.