神经退行性变
外显子组测序
基底神经节
疾病
病理
生物
基因
医学
神经科学
遗传学
中枢神经系统
突变
作者
Antonia Lefter,Iulia Mitrea,Dan Mitrea,Vasilica Plăiaşu,Aida M. Bertoli‐Avella,Christian Beetz,Liviu Cozma,Delia Tulbă,Cristina Mitu,Bogdan Ovidiu Popescu
出处
期刊:Neurocase
[Taylor & Francis]
日期:2021-11-02
卷期号:27 (6): 481-483
被引量:4
标识
DOI:10.1080/13554794.2021.2022703
摘要
Neurodegeneration with brain iron accumulation (NBIA) is a group of inherited disorders characterised by cerebral iron overload mainly in the basal ganglia. Mitochondrial membrane protein-associated neurodegeneration (MPAN) is a form of NBIA caused by pathogenic C19orf12 gene variants. We report on a Romanian patient with MPAN confirmed through exome sequencing, revealing a homozygous nonsense variant in the C19orf12 gene, NM_001031726.3: c.215T>G (p.Leu72*), that co-segregates with disease in tested relatives: the patient`s parents, younger brother and paternal uncle are heterozygous carriers. This is a novel disease-causing variant in the C19orf12 gene and the first reported MPAN case in a Romanian patient.
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