{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T09:31:10Z","timestamp":1791192670775,"version":"4.1.0"},"reference-count":0,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"3","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[1999,3]]},"abstract":"<jats:sec>\n                    <jats:title>Abstract.<\/jats:title>\n                    <jats:p>\n                      The objective of this study was to further characterize the clinical and immunopathologic features of heavy chain deposition disease (HCDD), a recently described entity. Four patients were diagnosed as having HCDD on a kidney biopsy. All presented with nodular glomerulosclerosis with deposition of \u03b31 heavy chains lacking C\n                      <jats:sub>H<\/jats:sub>\n                      1 epitopes, but without light chains. Two different patterns were observed in the serum. First, patients 1 and 2 had a circulating monoclonal IgG\u03bb containing a short \u03b31 heavy chain lacking C\n                      <jats:sub>H<\/jats:sub>\n                      1 epitopes, with an apparent molecular weight of 40 kD consistent with a complete C\n                      <jats:sub>H<\/jats:sub>\n                      1 deletion. Biosynthetic experiments also showed that the deleted heavy chain was produced in excess compared with light chains, and was secreted\n                      <jats:italic toggle=\"yes\">in vitro<\/jats:italic>\n                      together with half Ig molecules, although these abnormal components were not detected by Western blot analysis of whole serum. Second, patients 3 and 4 had a circulating monoclonal IgG1\u03bb with an apparently normal, nondeleted heavy chain subunit, but serum fractionation followed by immunoblotting revealed an isolated monoclonal \u03b31 chain lacking C\n                      <jats:sub>H<\/jats:sub>\n                      1 epitopes. These data strongly suggest that renal deposition of a C\n                      <jats:sub>H<\/jats:sub>\n                      1-deleted heavy chain circulating in low amounts in the serum as a free unassembled subunit is a major feature of HCDD. The C\n                      <jats:sub>H<\/jats:sub>\n                      1 deletion is most likely responsible for the premature secretion in blood of the heavy chain by a clone of plasma cells.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1681\/asn.v103519","type":"journal-article","created":{"date-parts":[[2021,4,28]],"date-time":"2021-04-28T04:46:54Z","timestamp":1619585214000},"page":"519-528","source":"Crossref","is-referenced-by-count":64,"title":["Nodular Glomerulosclerosis with Deposition of Monoclonal Immunoglobulin Heavy Chains Lacking CH1"],"prefix":"10.1681","volume":"10","author":[{"given":"BRUNO","family":"MOULIN","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"SOPHIE","family":"DERET","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"XAVIER","family":"MARIETTE","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"OLIVIER","family":"KOURILSKY","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"HIROKAZU","family":"IMAI","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"LUC","family":"DUPOUET","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"LUC","family":"MARCELLIN","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"ISABELLE","family":"KOLB","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"PIERRE","family":"AUCOUTURIER","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"JEAN-CLAUDE","family":"BROUET","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"PIERRE M.","family":"RONCO","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B\u00c9ATRICE","family":"MOUGENOT","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","container-title":["Journal of the American Society of Nephrology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ovid.com\/00001751-199903000-00010","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,7]],"date-time":"2026-08-07T19:24:51Z","timestamp":1786130691000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ovid.com\/00001751-199903000-00010"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1999,3]]},"references-count":0,"journal-issue":{"issue":"3","published-print":{"date-parts":[[1999]]}},"URL":"https:\/\/doi.org\/10.1681\/asn.v103519","relation":{},"ISSN":["1046-6673"],"issn-type":[{"value":"1046-6673","type":"print"}],"subject":[],"published":{"date-parts":[[1999,3]]}}}