{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T12:08:54Z","timestamp":1791893334445,"version":"4.3.4"},"reference-count":38,"publisher":"Oxford University Press (OUP)","issue":"2","license":[{"start":{"date-parts":[[2003,10,23]],"date-time":"2003-10-23T00:00:00Z","timestamp":1066867200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"funder":[{"DOI":"10.13039\/100000002","name":"NIH","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Oncology Research"},{"DOI":"10.13039\/100008216","name":"Jonsson Cancer Center","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100008216","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Molecular Biology Institute at UCLA"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2004,2,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Neutrophil-specific granule deficiency (SGD) is a rare, congenital disease characterized by atypical neutrophil structure and function, resulting in recurrent bacterial infections from early infancy. Homozygous recessive mutations in the CCAAT\/enhancer-binding protein \u025b (C\/EBP\u025b) gene were described in two of five SGD patients, indicating loss of C\/EBP\u025b function as the primary genetic defect in this disease. C\/EBP\u025b is expressed in murine and human macrophages. Macrophages from the C\/EBP\u025b-deficient mice show impaired differentiation, phagocytic activity, and transcription of macrophage-specific genes. To determine if monocyte\/macrophage cells are impacted in SGD, we analyzed phenotypic features of peripheral blood (PB) monocytes in a SGD individual lacking functional C\/EBP\u025b. Flow cytometric analysis of PB leukocytes revealed aberrant expression of CD45, CD11b, CD14, CD15, and CD16 on cells from the SGD individual. Also, the PB CD14+ cells from this individual, weakly stained for the monocyte-specific enzyme, nonspecific esterase, and electron microscopic examination, indicated morphologic differences between the SGD cells and those from normal controls. Serum interleukin (IL)-6 levels in the SGD individual during a severe bacterial infection were lower compared with levels in other non-SGD individuals with sepsis. In contrast, serum IL-8 levels were markedly elevated in the SGD individual compared with those of non-SGD individuals in sepsis. PB CD14+ cells from the SGD individual expressed higher IL-8 mRNA levels compared with normal controls in response to lipopolysaccharide and interferon-\u03b3. These phenotypic and functional alterations of PB monocytes in the SGD individual suggest that C\/EBP\u025b plays a critical role in monocyte\/macrophage development of humans and is consistent with observations in the murine system. This study implicates abnormalities in monocytes\/macrophages and neutrophils in the onset and development of SGD.<\/jats:p>","DOI":"10.1189\/jlb.0203063","type":"journal-article","created":{"date-parts":[[2003,10,28]],"date-time":"2003-10-28T07:44:52Z","timestamp":1067327092000},"page":"190-197","source":"Crossref","is-referenced-by-count":39,"title":["Phenotypic and functional alterations of peripheral blood monocytes in neutrophil-specific granule deficiency"],"prefix":"10.1093","volume":"75","author":[{"given":"Masaaki","family":"Shiohara","sequence":"first","affiliation":[{"name":"Departments of Pediatrics , Matsumoto , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adrian F","family":"Gombart","sequence":"additional","affiliation":[{"name":"Division of Hematology\/Oncology, Cedars-Sinai Medical Center , Los Angeles, California"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yukio","family":"Sekiguchi","sequence":"additional","affiliation":[{"name":"Departments of Pediatrics , Matsumoto , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eiko","family":"Hidaka","sequence":"additional","affiliation":[{"name":"Departments of Laboratory Medicine , Matsumoto , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susumu","family":"Ito","sequence":"additional","affiliation":[{"name":"Division of Blood Transfusion, Shinshu University School o Medicine , Matsumoto , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takashi","family":"Yamazaki","sequence":"additional","affiliation":[{"name":"Departments of Pediatrics , Matsumoto , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H Phillip","family":"Koeffler","sequence":"additional","affiliation":[{"name":"Division of Hematology\/Oncology, Cedars-Sinai Medical Center , Los Angeles, California"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Atsushi","family":"Komiyama","sequence":"additional","affiliation":[{"name":"Departments of Pediatrics , Matsumoto , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2003,10,23]]},"reference":[{"key":"2023033100212943900_","doi-asserted-by":"crossref","first-page":"478","DOI":"10.1056\/NEJM197402282900903","article-title":"An anomaly of neutrophil morphology with impaired function","volume":"290","author":"Strauss","year":"1974","journal-title":"N. 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