| Literature DB >> 31191205 |
Akella Radha Rama Devi1, Aparna Ganapathy2, Ashraf U Mannan2, Shefali Sabharanjak2, Shaik M Naushad3.
Abstract
Chromosome 1q42.12q42.2 deletions are documented as "disease causing" and show overlapping phenotypes depending on the genes involved in the deletion. In this report, we detected a 5.8-Mb deletion encompassing the chromosome 1q42.12q42.2 region in a 4-year-old boy with hypoplastic corpus callosum, epilepsy, developmental delay, microcephaly, cataract, cleft palate, and skeletal changes. The deletion was de novo. Genotype-phenotype correlations suggest that the major features of 1q42.12q42.2 microdeletion were attributed to the genes with a high probability of loss-of-function intolerance score in this deletion, namely LBR, ENAH, ACBD3, LIN9, ITPKB, CDC42BPA, ARF1, TAF5L, GALNT2, SPRTN, and EGLN1 along with GNPAT.Entities:
Keywords: 1q42.12q42.2 deletion; GNPAT; LBR; pLI score
Year: 2019 PMID: 31191205 PMCID: PMC6528072 DOI: 10.1159/000496079
Source DB: PubMed Journal: Mol Syndromol ISSN: 1661-8769