<prompt> Answer this question truthfullyCould you provide me with the context in which you want me to rephrase this statement? Do you want it in a more formal tone?  <response>: <reason> Hurler-Scheie syndrome is caused by mutations in the IDUA gene 4p16.3 leading to partial deficiency in the alpha-L-iduronidase enzyme and lysosomal accumulation of dermatan sulfate and heparan sulfate. Transmission is autosomal recessive. 