Novel C19orf12 loss-of-function variant leading to neurodegeneration with brain iron accumulation

被引:1
|
作者
Lefter, Antonia [1 ,2 ]
Mitrea, Iulia [1 ,2 ]
Mitrea, Dan [3 ]
Plaiasu, Vasilica [4 ]
Bertoli-Avella, Aida [5 ]
Beetz, Christian [5 ]
Cozma, Liviu [2 ,3 ]
Tulba, Delia [1 ,2 ]
Elena Mitu, Cristina [1 ]
Ovidiu Popescu, Bogdan [1 ,2 ,6 ]
机构
[1] Colentina Clin Hosp, Dept Neurol, Stefan Cel Mare St 19-21, Bucharest, Romania
[2] Carol Davila Univ Med & Pharm, Dept Clin Neurosci, Bucharest, Romania
[3] Neuroaxis Neurol Clin, Bucharest, Romania
[4] Alfred Rusescu Inst Mother & Child, Dept Clin Genet, Bucharest, Romania
[5] CENTOGENE GmbH, Rostock, Germany
[6] Victor Babes Natl Inst Pathol, Lab Mol Med, Bucharest, Romania
关键词
C19orf12; mitochondrial membrane protein-associated neurodegeneration; neurodegeneration with brain iron accumulation; KINASE-ASSOCIATED NEURODEGENERATION; LIPID-METABOLISM; DEFERIPRONE; EFFICACY; PROTEIN; SUBTYPE; SAFETY;
D O I
10.1080/13554794.2021.2022703
中图分类号
R74 [神经病学与精神病学];
学科分类号
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.
引用
收藏
页码:481 / 483
页数:3
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