PRRT2, a network stability gene

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PRRT2, a network stability gene

Specific paroxysmal disorders, namely benign infantile epilepsy (BFIE), kinesigenic dyskinesia (PKD), infantile convulsions and choreoathetosis (ICCA) and hemiplegic migraine (HM), are associated with mutations in the gene encoding for PRoline-Rich Transmembrane protein 2 (PRRT2; Figure 1A, 1B). PRRT2 is a neuronspecific protein expressed on neuronal membranes and at synapses, with a prevalent ...

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PRRT2 Mutations and PRRT2 Disorders

The recent discovery of PRRT2 mutations in Paroxysmal Kinesigenic Dyskinesia (PKD) has spurred a number of studies on PRRT2 mutations and PRRT2 disorders including PKD, Benign Familial Infantile Epilepsy (BFIE), and Infantile Convulsions with Choreoathetosis (ICCA). Mutated PRRT2 is also implicated in several other paroxysmal neurological disorders, indicating a wide phenotypic spectrum of PRRT...

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Role of PRRT2 in common paroxysmal neurological disorders: a gene with remarkable pleiotropy.

Mutations in the gene PRRT2 encoding proline-rich transmembrane protein 2 have recently been identified as the cause of three clinical entities: benign familial infantile epilepsy (BFIE), infantile convulsions with choreoathetosis (ICCA) syndrome, and paroxysmal kinesigenic dyskinesia (PKD). Patients with ICCA have both BFIE and PKD and families with ICCA may contain individuals who exhibit all...

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The PRRT2 knockout mouse recapitulates the neurological diseases associated with PRRT2 mutations

Heterozygous and rare homozygous mutations in PRoline-Rich Transmembrane protein 2 (PRRT2) underlie a group of paroxysmal disorders including epilepsy, kinesigenic dyskinesia episodic ataxia and migraine. Most of the mutations lead to impaired PRRT2 expression and/or function. Recently, an important role for PRTT2 in the neurotransmitter release machinery, brain development and synapse formatio...

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ژورنال

عنوان ژورنال: Oncotarget

سال: 2017

ISSN: 1949-2553

DOI: 10.18632/oncotarget.19506