The purpose of this study is to evaluate the safety and exploratory efficacy of GC1119 (recombinant human α-galactosidase A) for enzyme replacement therapy in Fabry disease patients.

Inclusion Criteria:

• Subjects with a current diagnosis of Fabry's disease
• Plasma α-gal activity of ≤ 1.5mnol/hr/ml and have a mutation in α-galactosidase A gene
• Males ≥ 16 years old
• Subjects capable of performing this clinical trial in an appropriate manner
• Informed consent form voluntarily signed by the subject(or his legally acceptable representative if the subject is under 20 years old) to participation in the study
• Agreement to contraception during the study period

Exclusion Criteria:

• Serum creatinine > 2.5mg/dl
• Subjects have a plan to kidney transplantation
• Subjects have undergone kidney transplantation
• Subjects are currently on dialysis
• Subjects have a clinically significant organic disease(cardiovascular, hepatic, pulmonary, neurologic, or renal disease)that in the opinion of the investigator would preclude participation in the trial
• Known life-threatening hypersensitivity(anaphylactic reaction) to α-galactosidase
• Treatment with another investigational product within 30days from the administration of study drug dosing or plans to be treated with another investigational product during the study period
• Known hypersensitivity to any of the ingredients of study drug(including excipients)
• Subjects need the medication of prohibited drug
• Alcoholism or drug addiction