LMNA gene and its involvement in Hutchinson-Gilford Progeria Syndrome (HGPS) and arteriosclerosis
    6.
    发明授权
    LMNA gene and its involvement in Hutchinson-Gilford Progeria Syndrome (HGPS) and arteriosclerosis 有权
    LMNA基因及其参与Hutchinson-Gilford Progeria综合征(HGPS)和动脉硬化

    公开(公告)号:US09115400B2

    公开(公告)日:2015-08-25

    申请号:US14025497

    申请日:2013-09-12

    IPC分类号: C07H21/04 C07K14/47 C12Q1/68

    摘要: Disclosed herein are point mutations in the LMNA gene that cause HGPS. These mutations activate a cryptic splice site within the LMNA gene, which leads to deletion of part of exon 11 and generation of a mutant Lamin A protein product that is 50 amino acids shorter than the normal protein. In addition to the novel Lamin A variant protein and nucleic acids encoding this variant, methods of using these molecules in detecting biological conditions associated with a LMNA mutation in a subject (e.g., HGPS, arteriosclerosis, and other age-related diseases), methods of treating such conditions, methods of selecting treatments, methods of screening for compounds that influence Lamin A activity, and methods of influencing the expression of LMNA or LMNA variants are also described. Oligonucleotides and other compounds for use in examples of the described methods are also provided, as are protein-specific binding agents, such as antibodies, that bind specifically to at least one epitope of a Lamin A variant protein preferentially compared to wildtype Lamin A, and methods of using such antibodies in diagnosis, treatment, and screening. Also provided are kits for carrying out the methods described herein.

    摘要翻译: 本文公开了导致HGPS的LMNA基因中的点突变。 这些突变激活LMNA基因内的隐性剪接位点,导致部分外显子11的缺失和产生比正常蛋白短50个氨基酸的突变Lamin A蛋白产物。 除了新型Lamin A变体蛋白和编码该变体的核酸之外,使用这些分子检测与受试者的LMNA突变相关的生物学条件(例如,HGPS,动脉硬化和其他与年龄有关的疾病)的方法, 还描述了治疗这些病症,选择治疗方法,筛选影响Lamin A活性的化合物的方法,以及影响LMNA或LMNA变体表达的方法。 还提供了用于所述方法的实施例的寡核苷酸和其它化合物,以及优选与野生型Lamin A相比特异性结合Lamin A变体蛋白的至少一个表位的蛋白质特异性结合剂,例如抗体,以及 在诊断,治疗和筛查中使用这些抗体的方法。 还提供了用于实施本文所述方法的试剂盒。

    Jam detection and safety device for jamming machinery
    8.
    发明授权
    Jam detection and safety device for jamming machinery 失效
    卡纸检测和干扰机械的安全装置

    公开(公告)号:US07493854B2

    公开(公告)日:2009-02-24

    申请号:US11593980

    申请日:2006-11-06

    IPC分类号: B30B15/18 F16P3/00

    摘要: The present disclosure concerns a jam detection and safety system for machines prone to jamming, such as a baler. In particular embodiments, the system is implemented in a horizontal baler having a shear bar and a hydraulic ram that advances material being baled past the shear bar into a compression chamber. The system includes a strain gage mounted on the shear bar and a controller that is electrically connected to the strain gage. The controller receives strain signals from the stage gage and compares the strain of the shear bar to a predetermined strain threshold corresponding to a jamming condition. If the strain exceeds the predetermined threshold, the controller automatically deactivates the ram, such as by disconnecting line power from the baler and/or by turning off the control power of the baler.

    摘要翻译: 本公开涉及用于容易发生卡纸的机器的卡纸检测和安全系统,例如打捆机。 在具体实施例中,该系统在具有剪切杆和液压柱塞的水平打捆机中实现,该打浆机将经过剪切杆打包的材料推进到压缩室中。 该系统包括安装在剪切杆上的应变计和电连接到应变计的控制器。 控制器从分级计接收应变信号,并将剪切杆的应变与对应于干扰条件的预定应变阈值进行比较。 如果应变超过预定阈值,则控制器自动停用冲头,例如通过断开打捆机的线路电源和/或关闭打包机的控制功率。