نتایج جستجو برای: dyt6 dystonia

تعداد نتایج: 6616  

Journal: :Parkinsonism & related disorders 2012
Alexander Schmidt Eckart Altenmüller Hans-Christian Jabusch André Lee Karin Wiegers Christine Klein Katja Lohmann

Focal dystonia is the most common form of primary dystonia characterized by involuntary twisting, abnormal postures and repetitive movements in a specific body part. Musician’s dystonia (MD) is a type of focal task-specific dystonia that presents with loss of voluntary motor control of extensively trained movements when a musician is playing his instrument. The pathophysiology of focal dystonia...

2014
Carlos Henrique F. Camargo Sarah Teixeira Camargos Salmo Raskin Francisco Eduardo C. Cardoso Hélio Afonso G. Teive

BACKGROUND Several genes associated with dystonia have been identified. A mutation in one of these, THAP1 (DYT6), is linked to isolated dystonia. The aim of this study was to assess the prevalence of THAP1 gene mutations and the clinical characteristics of patients with these mutations in a clinical population in Brazil. METHODS Seventy-four patients presenting with dystonia involving the cer...

Journal: :Movement disorders : official journal of the Movement Disorder Society 2009
Monica Bonetti Chiara Barzaghi Francesco Brancati Alessandro Ferraris Emanuele Bellacchio Alessandro Giovanetti Tamara Ialongo Giovanna Zorzi Carla Piano Martina Petracca Alberto Albanese Nardo Nardocci Bruno Dallapiccola Anna Rita Bentivoglio Barbara Garavaglia Enza Maria Valente

Mutations in the THAP1 gene on chromosome 8p21-p22 (DYT6 locus) have been recently reported as causative of autosomal dominant primary torsion dystonia (PTD) in four Amish-Mennonite families and in 12 additional probands of different ancestry. We sequenced the THAP1 gene in 158 patients with DYT1-negative PTD who had onset of symptoms below 30 years and/or positive family history. One sporadic ...

2014
Maria Stamelou Gavin Charlesworth Carla Cordivari Susanne A Schneider Georg Kägi Una-Marie Sheerin Ignacio Rubio-Agusti Amit Batla Henry Houlden Nicholas W Wood Kailash P Bhatia

Genes causing primary dystonia are rare. Recently, pathogenic mutations in the anoctamin 3 gene (ANO3) have been identified to cause autosomal dominant craniocervical dystonia and have been assigned to the dystonia locus dystonia-24 (DYT24). Here, we expand on the phenotypic spectrum of DYT24 and provide demonstrative videos. Moreover, tremor recordings were performed, and back-averaged electro...

2017
Francesca Aguilo Zuchra Zakirova Katie Nolan Ryan Wagner Rajal Sharma Megan Hogan Chengguo Wei Yifei Sun Martin J. Walsh Kevin Kelley Weijia Zhang Laurie J. Ozelius Pedro Gonzalez-Alegre Thomas P. Zwaka Michelle E. Ehrlich

THAP1 (THAP [Thanatos-associated protein] domain-containing, apoptosis-associated protein 1) is a ubiquitously expressed member of a family of transcription factors with highly conserved DNA-binding and protein-interacting regions. Mutations in THAP1 cause dystonia, DYT6, a neurologic movement disorder. THAP1 downstream targets and the mechanism via which it causes dystonia are largely unknown....

2018
Zuchra Zakirova Tomas Fanutza Justine Bonet Ben Readhead Weijia Zhang Zhengzi Yi Genevieve Beauvais Thomas P Zwaka Laurie J Ozelius Robert D Blitzer Pedro Gonzalez-Alegre Michelle E Ehrlich

Dystonia is characterized by involuntary muscle contractions. Its many forms are genetically, phenotypically and etiologically diverse and it is unknown whether their pathogenesis converges on shared pathways. Mutations in THAP1 [THAP (Thanatos-associated protein) domain containing, apoptosis associated protein 1], a ubiquitously expressed transcription factor with DNA binding and protein-inter...

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