The study revealed that skewed XCI was identified in 29% of the samples.A correlation was found between XCI patterns in the blood and other samples.Skewing was responsible for differences in GAL levels, clinical severity scores, worsening of cardiomyopathy, and kidney disease. Global clinical severity change was assessed using the MSSI and DS3.Overall, the study suggested that XCI can impact the clinical phenotype in individuals with Fabry disease. The gene discussed is GAL; the disease is Fabry disease.