Department of Pharmacology, University of Texas Southwestern Medical Center, Dallas, Texas 75390, USA.
J Biol Chem. 2012 Jan 27;287(5):3123-37. doi: 10.1074/jbc.M111.324350. Epub 2011 Dec 1.
Lipin-1 catalyzes the formation of diacylglycerol from phosphatidic acid. Lipin-1 mutations cause lipodystrophy in mice and acute myopathy in humans. It is heavily phosphorylated, and the yeast ortholog Pah1p becomes membrane-associated and active upon dephosphorylation by the Nem1p-Spo7p membrane complex. A mammalian ortholog of Nem1p is the C-terminal domain nuclear envelope phosphatase 1 (CTDNEP1, formerly "dullard"), but its Spo7p-like partner is unknown, and the need for its existence is debated. Here, we identify the metazoan ortholog of Spo7p, TMEM188, renamed nuclear envelope phosphatase 1-regulatory subunit 1 (NEP1-R1). CTDNEP1 and NEP1-R1 together complement a nem1Δspo7Δ strain to block endoplasmic reticulum proliferation and restore triacylglycerol levels and lipid droplet number. The two human orthologs are in a complex in cells, and the amount of CTDNEP1 is increased in the presence of NEP1-R1. In the Caenorhabditis elegans embryo, expression of nematode CTDNEP1 and NEP1-R1, as well as lipin-1, is required for normal nuclear membrane breakdown after zygote formation. The expression pattern of NEP1-R1 and CTDNEP1 in human and mouse tissues closely mirrors that of lipin-1. CTDNEP1 can dephosphorylate lipins-1a, -1b, and -2 in human cells only in the presence of NEP1-R1. The nuclear fraction of lipin-1b is increased when CTDNEP1 and NEP1-R1 are co-expressed. Therefore, NEP1-R1 is functionally conserved from yeast to humans and functions in the lipin activation pathway.
脂联素 1 催化从磷酸脂酰基转移酶到二酰基甘油的形成。脂联素 1 突变导致小鼠脂肪营养不良和人类急性肌病。它被高度磷酸化,酵母直系同源物 Pah1p 在被 Nem1p-Spo7p 膜复合物去磷酸化后成为膜相关并具有活性。Nem1p 的哺乳动物直系同源物是核包膜磷酸酶 1(CTDNEP1,以前称为“dullard”)的 C 末端结构域,但它的 Spo7p 样伴侣未知,其存在的必要性存在争议。在这里,我们鉴定了 Spo7p 的后生动物直系同源物,TMEM188,重新命名为核包膜磷酸酶 1 调节亚基 1(NEP1-R1)。CTDNEP1 和 NEP1-R1 共同补充 nem1Δspo7Δ 菌株以阻断内质网增殖并恢复三酰基甘油水平和脂滴数量。这两种人类直系同源物在细胞中形成复合物,并且在存在 NEP1-R1 的情况下 CTDNEP1 的量增加。在秀丽隐杆线虫胚胎中,线虫 CTDNEP1 和 NEP1-R1 的表达以及 lipin-1 的表达对于合子形成后核膜破裂是必需的。NEP1-R1 和 CTDNEP1 在人类和小鼠组织中的表达模式与 lipin-1 非常相似。CTDNEP1 仅在存在 NEP1-R1 的情况下才能在人类细胞中去磷酸化 lipin-1a、-1b 和 -2。当 CTDNEP1 和 NEP1-R1 共表达时,lipin-1b 的核部分增加。因此,NEP1-R1 从酵母到人类在功能上是保守的,并在 lipin 激活途径中发挥作用。