ATP11C is critical for the internalization of phosphatidylserine and differentiation of B lymphocytes.
ATP11C is critical for the internalization of phosphatidylserine and differentiation of B lymphocytes.
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质膜的亚室被认为是淋巴细胞反应的关键，但几乎没有遗传工具来测试它们的功能。在这里，我们描述了一种新的X连锁B细胞缺陷综合征，这种综合征是由P4 ATPase家族的成员Atp11c突变引起的，该家族被认为是在细胞膜的细胞质小叶中浓缩氨基磷脂的翻转酶。ATP11c缺陷降低了前B细胞的磷脂酰丝氨酸转移率，极大地减少了不依赖于bcl2抑制的凋亡或免疫球蛋白基因重排的前B和B细胞数量，并取消了对IL7转基因的前B细胞的扩增。注意到的其他异常仅有贫血、高胆红素血症和肝细胞癌。这些结果证实了磷脂转运与B淋巴细胞功能之间的密切联系。
Subcompartments of the plasma membrane are believed to be critical for lymphocyte responses but few genetic tools exist to test their function. Here we describe a new X-linked B cell deficiency syndrome in mice caused by mutations in Atp11c, a member of the P4 ATPase family thought to serve as flippases concentrating aminophospholipids in the cytoplasmic leaflet of cell membranes. Defective ATP11c decreased the rate of phosphatidylserine translocation in pro-B cells, greatly reduced pre-B and B cell numbers independent of Bcl2-inhibited apoptosis or immunoglobulin gene rearrangement and abolished pre-B cell expansion in response to an Il7 transgene. The only other abnormalities noted were anemia, hyperbilirubinemia and hepatocellular carcinoma. These results identify an intimate connection between phospholipid transport and B lymphocyte function.
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Dillon, SR;Mancini, M;Schlissel, MS
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