Abstract: The present invention relates to methods of preventing or treating a congenital myasthenic syndrome (CMS) in a subject, wherein the CMS is (a) congenital myasthenic syndrome associated with AChR deficiency or (b) fast-channel congenital myasthenic syndrome (FCCMS), the method comprising administering an effective amount of a DOK7 gene or a Dok-7 polypeptide, preferably a rAAV-DOK7 vector, to a subject in need thereof. The invention also relates to products for use in such methods.

Abstract: The present invention relates to a method for detecting antibodies against a target antigen in a sample which comprises contacting the sample with labelled target antigen, subjecting the sample to immunoprecipitation to precipitate antibodies in the sample and detecting the presence of antibodies against the target antigen in the sample by means of the presence of labelled target antigen in the immunoprecipitate, wherein the labelled target antigen is a fusion protein comprising the target antigen and a fluorescent protein label and the presence of labelled target antigen in the immunoprecipitate is detected by means of the fluorescence of the fluorescent label. The method is particularly suitable for use where the target antigen is an autoantigen and can also be used to identify autoantigens implicated in a particular autoimmune disorder by screening serum samples from patients with a clinical phenotype indicative or suggestive of an autoimmune disorder and suitable controls.

Abstract: A clock recovery system (10) for recovering an input data signal (14) clock. A rate detector (20) detects the input data signal bit rate and provides range signals (30a-c) specifying progressive ranges encompassing the bit rate. A frequency detector (22) provides a frequency error signal (32) based on frequency difference between the input data signal and a recovered clock signal (16). A phase detector (24) provides a phase error signal (34) based on the input data and recovered clock signals. A filter-controller (26) provides an oscillator driving signal (36) based on the range, frequency error, and phase error signals. An oscillator-divider (28) then provides the recovered clock signal based on the oscillator driving signal and at least some of the range signals. The phase detector, filter-controller, and oscillator-divider collectively thus form a phase locked loop. Optionally, the clock recovery system (10) may also provide a recovered data signal (18).

Abstract: A guitar string support insert located between the saddle block of a guitar tremolo and each string passing over the saddle block which is held thereto by a string retaining screw operated device in order to reduce string breakage.