ABCA3 is a member of the ATP-binding cassette (ABC) family of transport proteins and is required for perinatal respiratory adaptation. Monoclonal and polyclonal antibodies were generated against a recombinant human ABCA3 peptide and used to assess its expression in the developing lung and adult tissues. Immunostaining for ABCA3 was detected at highest levels in type II epithelial cells of the lung but was also noted in other organs including liver, stomach, kidney, adrenal, pancreas, trachea, and brain. In the fetal lung, ABCA3 staining and mRNA increased prior to birth. Like other surfactant protein genes, ABCA3 expression was induced by thyroid transcription factor-1 in vitro. ABCA3 was coexpressed with SP-B and proSP-C in type II epithelial cells. ABCA3 staining was detected surrounding large, intracellular organelles consistent with its association with lamellar bodies. In the human fetal lung, ABCA3 staining was not detected prior to 22-23 weeks of gestation, except in the presence of pulmonary inflammation. ABCA3 was detected in type II epithelial cells of the human lung from 28 weeks of gestation and thereafter. Postnatally, intense ABCA3 staining was observed in hyperplastic epithelial cells relining injured airways in infants with chronic lung disease. Localization and regulation of ABCA3 in the respiratory epithelium is consistent with its proposed role in surfactant homeostasis. The role of ABCA3 in extrapulmonary tissues and organs remains to be elucidated. This manuscript contains online supplemental material at (www.jhc.org). Please visit this article online to view these materials.

译文

:ABCA3是运输蛋白ATP结合盒(ABC)家族的成员,是围产期呼吸适应所必需的。产生了针对重组人ABCA3肽的单克隆和多克隆抗体,并用于评估其在发育中的肺和成人组织中的表达。在肺的II型上皮细胞中检测到最高水平的ABCA3免疫染色,但在其他器官(包括肝,胃,肾,肾上腺,胰腺,气管和脑)中也发现了免疫染色。在胎儿肺中,出生前ABCA3染色和mRNA增加。像其他表面活性剂蛋白基因一样,ABCA3表达在体外由甲状腺转录因子-1诱导。 ABCA3在II型上皮细胞中与SP-B和proSP-C共表达。在周围较大的细胞内细胞器中检测到ABCA3染色,这与它与层状体的结合相一致。在人胎肺中,除非存在肺部炎症,否则在妊娠22-23周之前未检测到ABCA3染色。从妊娠28周开始,在人肺的II型上皮细胞中检测到ABCA3。出生后,在患有慢性肺病的婴儿中,在衬砌受伤气道的增生上皮细胞中观察到了强烈的ABCA3染色。 ABCA3在呼吸道上皮细胞中的定位和调节与其在表面活性剂稳态中的拟议作用一致。 ABCA3在肺外组织和器官中的作用仍有待阐明。该手稿包含在线补充材料,网址为(www.jhc.org)。请在线访问本文以查看这些材料。

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