Gerding Wanda Maria, Koetting Judith, Epplen Jörg Thomas, Neusch Clemens
Human Genetics, Ruhr-University, MA 5/39, Bochum 44780, Germany.
Neuromuscul Disord. 2009 Oct;19(10):701-3. doi: 10.1016/j.nmd.2009.05.006. Epub 2009 Jun 21.
Hereditary motor-sensory neuropathy (HMSN) Type 1/CMT 1 is a disorder of the peripheral nervous system. The underlying genetic cause is heterogeneous, and mutations in LITAF (Lipopolysaccharide-induced TNF-alpha factor) represent a rare cause of CMT Type 1. In this report, a novel missense mutation is presented in the LITAF gene (c.430G>A p.V144M) in a German CMT family exhibiting typical electrophysiological features of a demyelinating neuropathy with conduction blocks and variable age at onset. Molecular genetic characterization of demyelinating HMSN should therefore include screening of the LITAF gene if typical signs of a non-homogenous demyelinating neuropathy combined with dominant familial occurrence are evident.
遗传性运动感觉神经病1型(HMSN)/遗传性多发性神经病1型(CMT 1)是一种周围神经系统疾病。其潜在的遗传病因具有异质性,脂多糖诱导的肿瘤坏死因子-α因子(LITAF)基因突变是CMT 1型的一种罕见病因。在本报告中,一个德国CMT家系的LITAF基因出现了一个新的错义突变(c.430G>A p.V144M),该家系表现出典型的脱髓鞘性神经病的电生理特征,伴有传导阻滞和发病年龄各异。因此,如果非均一性脱髓鞘性神经病的典型体征与显性家族性发病相结合很明显,脱髓鞘性HMSN的分子遗传学特征分析应包括对LITAF基因的筛查。