Interleukin-2 receptor gamma chain mutation results in X-linked severe combined immunodeficiency in humans
Article Abstract:
The interleukin-2 receptor (IL-2R) gamma chain is shown to have a mutation that leads to X-linked severe combined immunodeficiency (XSCID) in humans. This is proven through in situ chromosomal hybridization with particular plasmids. Thus, the relation between the IL-2R gamma gene and XSCID has important implications for prenatal and postnatal diagnosis, carrier female detection and gene therapy for XSCID. In addition, valuable insight is given to B cells, thymic maturation of bone marrow-derived precursor human T cells and the impact of other components of the IL-2-IL-2 receptor system.
Publication Name: Cell
Subject: Biological sciences
ISSN: 0092-8674
Year: 1993
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Crystal structure of a vertebrate smooth muscle myosin motor domain and its complex with the essential light chain: visualization of the pre-power stroke state
Article Abstract:
A study was conducted on crystal structures of an expressed chicken smooth muscle myosin motor domain (MD) with MgADP-AlF4- and an MD-essential light chain complex with MgADP-AlF4- and MgADP-Befx nucelotide analogs bound in the active site. Results showed that the MgADP-AlF4- and MgADP-Befx structures are similar in terms of binding to the actin. Moreover, a potential displacement of about 10 nanometer can be obtained during the power stroke.
Publication Name: Cell
Subject: Biological sciences
ISSN: 0092-8674
Year: 1998
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The strength of receptor signaling is centrally controlled through a cooperative loop between Ca(super 2+) and an oxidant signal
Article Abstract:
The Ca(super 2+) and reactive oxygen intermediates is generated upon BCR activation rapidly engage in a cooperative interaction that acts in a feedback manner to amplify the early signal generated. The existence of a positive feedback loop between receptor activation-dependent Ca(sub i)(super 2+) and ROS that regulates the BCR signal strength right at its point of origin is identified.
Publication Name: Cell
Subject: Biological sciences
ISSN: 0092-8674
Year: 2005
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