MFN2-related Charcot-Marie-Tooth Disease with Atypical Ocular Manifestations.
Charcot-Marie-Tooth disease
MFN2
astigmatism
cataracts
visual impairment
Journal
Internal medicine (Tokyo, Japan)
ISSN: 1349-7235
Titre abrégé: Intern Med
Pays: Japan
ID NLM: 9204241
Informations de publication
Date de publication:
15 Dec 2021
15 Dec 2021
Historique:
pubmed:
15
6
2021
medline:
18
12
2021
entrez:
14
6
2021
Statut:
ppublish
Résumé
We herein describe a Charcot-Marie-Tooth disease (CMT) family with a MFN2 mutation with atypical ocular manifestations. The proband, his mother, his third daughter, and his deceased maternal grandfather all had symptoms of CMT and a visual impairment (either cataracts or severe astigmatism). On whole-exome sequencing for the proband having CMT and congenital cataracts, we identified a c.314C>T (p.Thr105Met) mutation in MFN2, but no mutation in the causative genes associated with cataracts. This missense mutation in MFN2 co-segregated with CMT and the atypical ocular manifestations in this family. The findings of this study might help to expand the clinical phenotype of heterogeneous MFN2-related CMT.
Identifiants
pubmed: 34121014
doi: 10.2169/internalmedicine.7463-21
pmc: PMC8758439
doi:
Substances chimiques
Mitochondrial Proteins
0
GTP Phosphohydrolases
EC 3.6.1.-
MFN2 protein, human
EC 3.6.1.-
Types de publication
Case Reports
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
3969-3974Références
J Cell Biol. 2007 Feb 12;176(4):405-14
pubmed: 17296794
JAMA Neurol. 2014 Aug;71(8):1036-42
pubmed: 24957169
Cold Spring Harb Mol Case Stud. 2020 Jun 12;6(3):
pubmed: 32532879
J Neurol Sci. 2015 Sep 15;356(1-2):7-18
pubmed: 26143526
J Peripher Nerv Syst. 2016 Mar;21(1):38-44
pubmed: 26801520
Neuromolecular Med. 2006;8(1-2):63-74
pubmed: 16775367
Brain. 2006 Aug;129(Pt 8):2103-18
pubmed: 16835246
Neurology. 2003 Feb 25;60(4):696-9
pubmed: 12601114
Brain. 2012 Jan;135(Pt 1):23-34
pubmed: 22189565
Sci Rep. 2018 Jan 24;8(1):1522
pubmed: 29367651
Neurology. 2005 Jul 26;65(2):197-204
pubmed: 16043786
Medicine (Baltimore). 2016 Apr;95(16):e3453
pubmed: 27100445
Neurology. 2011 May 17;76(20):1690-6
pubmed: 21508331
Nat Genet. 2004 May;36(5):449-51
pubmed: 15064763
Neurology. 2005 Aug 9;65(3):496-7
pubmed: 16087932
J Peripher Nerv Syst. 2017 Sep;22(3):191-199
pubmed: 28660751