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Arch. endocrinol. metab. (Online) ; 64(5): 559-566, Sept.-Oct. 2020. tab, graf
Article in English | LILACS | ID: biblio-1131124

ABSTRACT

ABSTRACT Objective: Our aim is to establish genetic diagnosis of congenital generalized lipodystrophy (CGL) using targeted massively parallel sequencing (MPS), also known as next-generation sequencing (NGS). Subjects and methods: Nine unrelated individuals with a clinical diagnosis of CGL were recruited. We used a customized panel to capture genes related to genetic lipodystrophies. DNA libraries were generated, sequenced using the Illumina MiSeq, and bioinformatics analysis was performed. Results: An accurate genetic diagnosis was stated for all nine patients. Four had pathogenic variants in AGPAT2 and three in BSCL2. Three large homozygous deletions in AGPAT2 were identified by copy-number variant analysis. Conclusions: Although we have found allelic variants in only 2 genes related to CGL, the panel was able to identify different variants including deletions that would have been missed by Sanger sequencing. We believe that MPS is a valuable tool for the genetic diagnosis of multi-genes related diseases, including CGL.


Subject(s)
Humans , GTP-Binding Protein gamma Subunits/genetics , Lipodystrophy, Congenital Generalized/diagnosis , Lipodystrophy, Congenital Generalized/genetics , Lipodystrophy/diagnosis , Lipodystrophy/genetics , Alleles , High-Throughput Nucleotide Sequencing , Mutation/genetics
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