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Save in Desktop File name My First MMC Save as type Microsoft Management Console Files (*msc)

encryption or data backup) must request permission from individual device manufacturers in addition to the scrutiny placed on applications that request Restricted capabilities The three Manufacturer capabilities are listed in Table 7-8

Our understanding of the complex pathophysiology of the CMS has evolved over the past 30 years Although many have contributed, much of our knowledge derives from the efforts of Andrew Engel and colleagues at the Mayo Clinic163,174,185,426 In vitro electrophysiological recordings from motor points of intercostal muscle including patch-clamp experiments of single AChR channels have allowed analysis of their kinetic properties Ultrastructural imaging of nerve terminals, the synaptic cleft, and the muscle end plates have provided insight into normal and abnormal structure of the NMJ in these disorders Formulation of the knowledge gained by these interventions has translated into the identi cation, location, and function of the proteins involved in normal and abnormal NMT In some cases, this has provided the insight allowing identi cation of the genes responsible for a number of these CMS In the past 20 years, the number of recognized CMS has expanded from three to at least 10163 Understanding the pathophysiology of at least some of the CMS syndromes requires an explanation of the phenomenon of AChR desensitization This phenomenon refers to AChR unresponsiveness developing in response to excessive temporal and spatial ACh exposure with resultant failure of EPP generation356,552 555 A general overview of the pathophysiology of speci c CMS syndromes follows

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Choline acetyltransferase de ciency This enzyme is responsible for ACh synthesis, resynthesis, and repackaging Defects result in the inability to maintain a normal ACh content within individual vesicles in response to the stress of repetitive stimulation, typically delivered at a frequency of 10 Hz MEPP and EPP amplitudes are normal initially but decline in time as resynthesis is

required to replenish ACh stores556 Further support for this mechanism comes from similar synaptic responses to hemicholinium whose action is to block choline reuptake into the presynaptic terminal191,557 Paucity of synaptic vesicles and reduced quantal release The underlying cause of this syndrome is unknown Ultrastructural imaging reveals a synaptic vesicle population that is reduced by a factor of 4 in comparison to normal and a proportionate reduction in quantal content

b) Click Save to continue (Don t exit the MMC!) c) Notice in the upper-left corner of the open window that the name has changed d) Find the new icon that s been created on the desktop This icon, which bears the same name as your new MMC, will enable you to access the MMC in the future with just a double-click of the mouse

The capabilities for an executable are stored within the image file header This defines the capabilities that a successfully loaded process will possess It is the responsibility of the software install process to validate that the capabilities an install package s executables possess do not exceed those allowed based on the assigned trust level The capabilities for a dynamic library are also defined within the file header However, they do not have a direct impact on the capabilities of the resultant process They indicate what capabilities the library is allowed to execute with, not necessarily those that it will execute with In order to be successfully loaded into a live process, the dynamic link library (DLL) must have a set of capabilities comprising a superset of the capabilities required by the executable image As a result, most general-use shared libraries are defined with an almost complete set of capabilities all except for manufacturer capabilities

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