Department of Pediatrics, McGill University, Montreal Children's Hospital, Montreal, Quebec, Canada.
Dev Biol. 2010 May 1;341(1):154-66. doi: 10.1016/j.ydbio.2010.02.019. Epub 2010 Feb 21.
During vesicular transport between the endoplasmic reticulum and the Golgi, members of the TMED/p24 protein family form hetero-oligomeric complexes that facilitate protein-cargo recognition as well as vesicle budding. In addition, they regulate each other's level of expression. Despite analyses of TMED/p24 protein distribution in mammalian cells, yeast, and C. elegans, little is known about the role of this family in vertebrate embryogenesis. We report the presence of a single point mutation in Tmed2/p24beta(1) in a mutant mouse line, 99J, identified in an ENU mutagenesis screen for recessive developmental abnormalities. This mutation does not affect Tmed2/p24beta(1) mRNA levels but results in loss of TMED2/p24beta(1) protein. Prior to death at mid-gestation, 99J homozygous mutant embryos exhibit developmental delay, abnormal rostral-caudal elongation, randomized heart looping, and absence of the labyrinth layer of the placenta. We find that Tmed2/p24beta(1) is normally expressed in tissues showing morphological defects in 99J mutant embryos and that these affected tissues lack the TMED2/p24beta(1) oligomerization partners, TMED7/p24gamma(3) and TMED10/p24delta(1). Our data reveal a requirement for TMED2/p24beta(1) protein in the morphogenesis of the mouse embryo and placenta.
在内质网和高尔基体之间的小泡运输过程中,TMED/p24 蛋白家族的成员形成异源寡聚复合物,促进蛋白质货物的识别以及小泡出芽。此外,它们还调节彼此的表达水平。尽管对哺乳动物细胞、酵母和秀丽隐杆线虫中 TMED/p24 蛋白分布进行了分析,但对该家族在脊椎动物胚胎发生中的作用知之甚少。我们报告了在ENU 诱变筛选隐性发育异常的突变小鼠品系 99J 中存在 TMED2/p24beta(1) 的单个点突变。该突变不影响 Tmed2/p24beta(1)mRNA 水平,但导致 TMED2/p24beta(1)蛋白丢失。在中期死亡之前,99J 纯合突变体胚胎表现出发育迟缓、异常的头尾伸长、心脏环化随机化和胎盘迷路层缺失。我们发现 Tmed2/p24beta(1)在 99J 突变体胚胎中出现形态缺陷的组织中正常表达,并且这些受影响的组织缺乏 TMED2/p24beta(1)寡聚化伴侣 TMED7/p24gamma(3)和 TMED10/p24delta(1)。我们的数据揭示了 TMED2/p24beta(1)蛋白在小鼠胚胎和胎盘形态发生中的必要性。