{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T14:21:21Z","timestamp":1790173281294,"version":"4.1.0"},"reference-count":47,"publisher":"Oxford University Press (OUP)","issue":"4","license":[{"start":{"date-parts":[[2014,4,1]],"date-time":"2014-04-01T00:00:00Z","timestamp":1396310400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2014,4,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Heat shock proteins HSPA4L and HSPA4 are closely related members of the HSP110 family and act as cochaperones. We generated Hspa4l\u2212\/\u2212 \u00a0Hspa4\u2212\/\u2212 mice to investigate a functional complementarity between HSPA4L and HSPA4 during embryonic development. Hspa4l\u2212\/\u2212 \u00a0Hspa4\u2212\/\u2212 embryos exhibited marked pulmonary hypoplasia and neonatal death. Compared with lungs of wild-type, Hspa4l\u2212\/\u2212, and Hspa4\u2212\/\u2212 embryos, Hspa4l\u2212\/\u2212 \u00a0Hspa4\u2212\/\u2212 lungs were characterized by diminished saccular spaces and increased mesenchymal septa. Mesenchymal hypercellularity was determined to be due to an increased cell proliferation index and decreased cell death. A significant increase in expression levels of prosurvival protein B cell leukemia\/lymphoma 2 may be the cause for inhibition of apoptotic process in lungs of Hspa4\u2212\/\u2212 \u00a0Hspa4l\u2212\/\u2212 embryos. Accumulation of glycogen and diminished expression of surfactant protein B, prosurfactant protein C, and aquaporin 5 in saccular epithelium suggested impaired maturation of type II and type I pneumocytes in the Hspa4l\u2212\/\u2212 \u00a0Hspa4\u2212\/\u2212 lungs. Further experiments showed a significant accumulation of ubiquitinated proteins in the lungs of Hspa4l\u2212\/\u2212 \u00a0Hspa4\u2212\/\u2212 embryos, indicating an impaired chaperone activity. Our study demonstrates that HSPA4L and HSPA4 collaborate in embryonic lung maturation, which is necessary for adaptation to air breathing at birth.<\/jats:p>","DOI":"10.1165\/rcmb.2013-0132oc","type":"journal-article","created":{"date-parts":[[2013,8,27]],"date-time":"2013-08-27T15:04:26Z","timestamp":1377615866000},"page":"817-824","source":"Crossref","is-referenced-by-count":18,"title":["Respiratory Distress and Early Neonatal Lethality in\n                    <i>Hspa4l<\/i>\n                    \/\n                    <i>Hspa4<\/i>\n                    Double-Mutant Mice"],"prefix":"10.1093","volume":"50","author":[{"given":"Belal A.","family":"Mohamed","sequence":"first","affiliation":[{"name":"Institute of Human Genetics, and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amal Z.","family":"Barakat","sequence":"additional","affiliation":[{"name":"Institute of Human Genetics, and"},{"name":"National Research Centre, Cairo, Egypt"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Torsten","family":"Held","sequence":"additional","affiliation":[{"name":"Institute of Human Genetics, and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Manar","family":"Elkenani","sequence":"additional","affiliation":[{"name":"Institute of Human Genetics, and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christian","family":"M\u00fchlfeld","sequence":"additional","affiliation":[{"name":"Institute of Functional and Applied Anatomy, Hannover Medical School, Hannover, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J\u00f6rg","family":"M\u00e4nner","sequence":"additional","affiliation":[{"name":"Department of Anatomy and Embryology, Georg-August-University of G\u00f6ttingen, G\u00f6ttingen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ibrahim M.","family":"Adham","sequence":"additional","affiliation":[{"name":"Institute of Human 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