Deleterious mutation inGPR88is associated with chorea, speech delay, and learning disabilities

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Deleterious mutation in GPR88 is associated with chorea, speech delay, and learning disabilities

OBJECTIVE To identify the underlying molecular basis of a familial developmental disorder characterized by chorea, marked speech delay, and learning difficulties in 4 sisters from a consanguineous family. METHODS Whole-exome analysis of DNA of the 2 older patients followed by Sanger sequencing of the mutated exon in all family members. RESULTS A homozygous deleterious mutation, p.C291X, was...

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Mutation in Huntington's chorea.

Huntington's chorea is inherited as an autosomal dominant and in the majority of published pedigrees the disease appears regularly in every generation. If the information is available, the disease can sometimes be traced through many generations over a considerable span of time. Investigation of the original family group described by Huntington has made it possible to trace their origin to the ...

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Chorea-Ballismus Associated with Hyperglycemia.

Chorea-ballismus which is a rare complication of nonketotic hyperglycemia may be the first symptom of type 2 diabetes mellitus. In this paper, we present two patients, who had involuntary movements and were diagnosed as having ballismus-chorea associated with nonketotic hyperglycemia. While one of the patients was not diagnosed with diabetes mellitus, the other one did not administer insulin th...

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Chorea associated with nonketotic hyperglycemia

INTRODUCTION This is a unique case of nonketotic hyperglycemic (NKH) chorea in 84-year-old Asian woman. The patient had a history of type 2 diabetes mellitus more than 30 years, but had a poor control of blood sugar. She complained of acute onset of bilateral limb involuntary activities, and being easy to fall within a week. Laboratory testing disclosed hyperglycemia (669 mg/dL), glycated hemog...

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Perspective: Spontaneous Deleterious Mutation.

Mildly deleterious mutation has been invoked as a leading explanation for a diverse array of observations in evolutionary genetics and molecular evolution and is thought to be a significant risk of extinction for small populations. However, much of the empirical evidence for the deleterious-mutation process derives from studies of Drosophila melanogaster, some of which have been called into que...

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ژورنال

عنوان ژورنال: Neurology Genetics

سال: 2016

ISSN: 2376-7839

DOI: 10.1212/nxg.0000000000000064