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Lysosomal Phospholipase A2 and Phospholipidosis

机译：溶酶体磷脂酶A2和磷脂血症

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A lysosomal phospholipase A2, LPLA2, was recently characterized and shown to have substrate specificity for phosphatidylcholine and phosphatidylethanolamine. LPLA2 is ubiquitously expressed but is most highly expressed in alveolar macrophages. Double conditional gene targeting was employed to elucidate the function of LPLA2. LPLA2-deficient mice (Lpla2?/?) were generated by the systemic deletion of exon 5 of the Lpla2 gene, which encodes the lipase motif essential for the phospholipase A2 activity. The survival of the Lpla2?/? mice was normal. Lpla2?/? mouse mating pairs yielded normal litter sizes, indicating that the gene deficiency did not impair fertility or fecundity. Alveolar macrophages from wild-type but not Lpla2?/? mice readily degraded radiolabeled phosphatidylcholine. A marked accumulation of phospholipids, in particular phosphatidylethanolamine and phosphatidylcholine, was found in the alveolar macrophages, the peritoneal macrophages, and the spleens of Lpla2?/? mice. By 1 year of age, Lpla2?/? mice demonstrated marked splenomegaly and increased lung surfactant phospholipid levels. Ultrastructural examination of Lpla2?/? mouse alveolar and peritoneal macrophages revealed the appearance of foam cells with lamellar inclusion bodies, a hallmark of cellular phospholipidosis. Thus, a deficiency of lysosomal phospholipase A2 results in foam cell formation, surfactant lipid accumulation, splenomegaly, and phospholipidosis in mice.
机译：最近对溶酶体磷脂酶A2 LPLA2进行了表征，显示对磷脂酰胆碱和磷脂酰乙醇胺具有底物特异性。 LPLA2普遍存在，但在肺泡巨噬细胞中表达最高。使用双重条件基因靶向来阐明LPLA2的功能。 LPLA2缺陷小鼠（ Lpla2 ？/？）是通过系统性删除 Lpla2 基因的外显子5产生的，该基因编码脂肪酶基序对磷脂酶A2活性至关重要。 Lpla2 ？/？小鼠的存活是正常的。 Lpla2 ？/？小鼠交配对产仔数正常，表明该基因缺陷不会损害生育力或繁殖力。来自野生型但不是 Lpla2 ？/？小鼠的肺泡巨噬细胞很容易降解放射性标记的磷脂酰胆碱。在肺泡巨噬细胞，腹膜巨噬细胞和 Lpla2 α/β小鼠的脾脏中发现了明显的磷脂堆积，尤其是磷脂酰乙醇胺和磷脂酰胆碱。到1岁时， Lpla2 ？/？小鼠表现出明显的脾肿大和肺表面活性物质磷脂水平升高。对 Lpla2 ？/？小鼠肺泡和腹膜巨噬细胞的超微结构检查显示，泡沫细胞带有层状包涵体，这是细胞磷脂病的标志。因此，溶酶体磷脂酶A2的缺乏导致小鼠中泡沫细胞形成，表面活性剂脂质积累，脾肿大和磷脂病。

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