【摘 要】
：
Fabry disease(FD) is a rare lysosomal storage disorder that results from the lack of or deficiency of α-galactosidase A.Malfunctioning of α-galactosidase A fails to metabolize trihexosylceramides(THC)
【机 构】
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Deparment of Chemistry,Yonsei University,Seoul,120-749,Korea
【出 处】
：
43rd International Symposium on High Performance Liquid Phas
论文部分内容阅读
Fabry disease(FD) is a rare lysosomal storage disorder that results from the lack of or deficiency of α-galactosidase A.Malfunctioning of α-galactosidase A fails to metabolize trihexosylceramides(THC) and fatty substances,and ends up altering lipid profiles within various organs.Fabry patients receive enzyme replacement therapy(ERT) in an attempt to delay any progression and deterioration of FD and even though it is purported to reduce deposits of excessive lipids(especially THC),the reports on specific types of lipids that are affected by ERT are scarce.
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