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With the rapid development of molecular genetic testing, Alport syndrome causes have been restricted mostly to variants in the <jats:italic>COL4A5<\/jats:italic> or <jats:italic>COL4A3<\/jats:italic>\/<jats:italic>COL4A4<\/jats:italic> genes. Moreover, a broad range of genetic contributors in the complement and complement-regulating proteins are definitely implicated in the pathogenesis of C3GN.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Methods<\/jats:title>\n                <jats:p>We sought a family with persistent microscopic haematuria associated with renal failure. Clinicopathologic and follow-up data were obtained, and molecular genetic testing was used to screen for pathogenic variants.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Results<\/jats:title>\n                <jats:p>We describe a three-generation family with Alport syndrome showing a dominant maternal inheritance. Notably, renal biopsy showed the concurrent histological evidence of C3GN in the proband harbouring an uncommon heterozygous variation in <jats:italic>CFHR5<\/jats:italic>, c.508G\u2009&gt;\u2009A. The alteration leads to replacement of a highly conserved residue at position 170 of the \u03b2-strand subunit of <jats:italic>CFHR5<\/jats:italic> (p.Val170Met). In silico analysis showed that the variation was predicted to deregulate complement activation by altering the structural properties and enhancing C3b binding capacity to compete with Complement Factor H (CFH), which was in line with experimental data previously published.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusions<\/jats:title>\n                <jats:p>The comorbidity findings between Alport syndrome and C3GN indicate an underlying overlap and require further study.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s40001-021-00543-5","type":"journal-article","created":{"date-parts":[[2021,7,8]],"date-time":"2021-07-08T10:03:12Z","timestamp":1625738592000},"update-policy":"http:\/\/dx.doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Co-existence of Alport syndrome and C3 glomerulonephritis in a proband with family history"],"prefix":"10.1186","volume":"26","author":[{"given":"Yin","family":"Ding","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xuanli","family":"Tang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yuanyuan","family":"Du","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hongyu","family":"Chen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dongrong","family":"Yu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bin","family":"Zhu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bohan","family":"Yuan","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2021,7,8]]},"reference":[{"key":"543_CR1","doi-asserted-by":"publisher","first-page":"407","DOI":"10.1057\/jphp.2010.30","volume":"31","author":"BC Liu","year":"2010","unstructured":"Liu BC, He L, He G, et al. 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Written informed consent forms for participation in the study were obtained, where participants are children (under 16\u00a0years old) from their parent.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical approval and consent to participate"}},{"value":"Not applicable.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare that they have no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"71"}}