ジャーナル: J Cell Sci / 年: 2021 タイトル: A lamin A/C variant causing striated muscle disease provides insights into filament organization. 著者: Rafael Kronenberg-Tenga / Meltem Tatli / Matthias Eibauer / Wei Wu / Ji-Yeon Shin / Gisèle Bonne / Howard J Worman / Ohad Medalia / 要旨: The gene encodes the A-type lamins, which polymerize into ∼3.5-nm-thick filaments and, together with B-type lamins and associated proteins, form the nuclear lamina. Mutations in cause a wide ...The gene encodes the A-type lamins, which polymerize into ∼3.5-nm-thick filaments and, together with B-type lamins and associated proteins, form the nuclear lamina. Mutations in cause a wide variety of pathologies. In this study, we analyzed the nuclear lamina of embryonic fibroblasts from mice, which develop cardiomyopathy and muscular dystrophy. Although the organization of the lamina appeared unaltered, there were changes in chromatin and B-type lamin expression. An increase in nuclear size and consequently a relative reduction in heterochromatin near the lamina allowed for a higher resolution structural analysis of lamin filaments using cryo-electron tomography. This was most apparent when visualizing lamin filaments and using a nuclear extraction protocol. Averaging of individual segments of filaments in mouse fibroblasts resolved two polymers that constitute the mature filaments. Our findings provide better views of the organization of lamin filaments and the effect of a striated muscle disease-causing mutation on nuclear structure.
EMPIAR-10601 (タイトル: A lamin A/C variant causing striated muscle disease provides insights into filament organization Data size: 2.7 Data #1: Tilt-series of LmnaH222P/H222P revealing lamin meshwork in nuclease treated and FIB-milled nucleus [tilt series])
MEF cell carrying homozygous H222P mutation in Lmna.
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集束イオンビーム - 装置: OTHER / 集束イオンビーム - イオン: OTHER / 集束イオンビーム - 電圧: 30 kV / 集束イオンビーム - 電流: 0.24 nA / 集束イオンビーム - 時間: 3600 sec. / 集束イオンビーム - 温度: 128 K / 集束イオンビーム - Initial thickness: 4000 nm / 集束イオンビーム - 最終 厚さ: 200 nm 集束イオンビーム - 詳細: The value given for _emd_sectioning_focused_ion_beam.instrument is Zeiss Auriga. This is not in a list of allowed values {'DB235', 'OTHER'} so OTHER is written into the XML file.
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電子顕微鏡法
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FEI TITAN KRIOS
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フィルム・検出器のモデル: GATAN K2 SUMMIT (4k x 4k) 平均電子線量: 3.7 e/Å2