Literature DB >> 27078007

Recessive Mutation in a Nuclear-Encoded Mitochondrial tRNA Synthetase Associated With Infantile Cataract, Congenital Neurotrophic Keratitis, and Orbital Myopathy.

Samir Jabbour1, Mona Harissi-Dagher.   

Abstract

PURPOSE: To report the ocular findings of a rare case of mutation in the nuclear-encoded mitochondrial aminoacyl-tRNA synthetase IARS2.
METHODS: A 33-year-old woman known for infantile cataracts, growth hormone deficiency, sensory neuropathy, sensorineural hearing loss, and skeletal dysplasia was referred to us for multiple failed corneal grafts and severe eye dryness.
RESULTS: The patient was found to have neurotrophic keratitis and corneal opacification.
CONCLUSIONS: Patients with this very rare mutation present with a myriad of ocular findings, including infantile cataract, neurotrophic keratitis, corneal opacification, and orbital myopathy.

Entities:  

Mesh:

Substances:

Year:  2016        PMID: 27078007     DOI: 10.1097/ICO.0000000000000847

Source DB:  PubMed          Journal:  Cornea        ISSN: 0277-3740            Impact factor:   2.651


  4 in total

1.  Neurotrophic Keratopathy in Systemic Diseases: A Case Series on Patients Treated With rh-NGF.

Authors:  Alessandro Meduri; Giovanni William Oliverio; Antonio Valastro; Claudia Azzaro; Umberto Camellin; Francesco Franchina; Leandro Inferrera; Anna Roszkowska; Pasquale Aragona
Journal:  Front Med (Lausanne)       Date:  2022-05-30

2.  RNAi-mediated IARS2 knockdown inhibits proliferation and promotes apoptosis in human melanoma A375 cells.

Authors:  Dongmei Ma; Song Li; Xiaojuan Nie; Lamei Chen; Nan Chen; Dongsheng Hou; Xiuhong Liu; Binbin Gao
Journal:  Oncol Lett       Date:  2020-05-29       Impact factor: 2.967

3.  Expanding the clinical phenotype of IARS2-related mitochondrial disease.

Authors:  Barbara Vona; Reza Maroofian; Emanuele Bellacchio; Maryam Najafi; Kyle Thompson; Ahmad Alahmad; Langping He; Najmeh Ahangari; Abolfazl Rad; Sima Shahrokhzadeh; Paulina Bahena; Falk Mittag; Frank Traub; Jebrail Movaffagh; Nafise Amiri; Mohammad Doosti; Reza Boostani; Ebrahim Shirzadeh; Thomas Haaf; Daria Diodato; Miriam Schmidts; Robert W Taylor; Ehsan Ghayoor Karimiani
Journal:  BMC Med Genet       Date:  2018-11-12       Impact factor: 2.103

Review 4.  Skeletal Phenotypes Due to Abnormalities in Mitochondrial Protein Homeostasis and Import.

Authors:  Tian Zhao; Caitlin Goedhart; Gerald Pfeffer; Steven C Greenway; Matthew Lines; Aneal Khan; A Micheil Innes; Timothy E Shutt
Journal:  Int J Mol Sci       Date:  2020-11-06       Impact factor: 5.923

  4 in total

北京卡尤迪生物科技股份有限公司 © 2022-2023.