An Extension of a Phase 1/2, Open Label, Dose Ranging Study of PRX-102 in Adult Fabry Patients

Patients will be enrolled into one of three PRX-102 dosing groups (0.2 mg/kg, 1.0 mg/kg, 2.0
mg/kg) to receive the same dose they had received in the Phase 1/2 study, PB-102-F01, and
will continue to receive intravenous infusions of PRX-102 every 2 weeks for 38 weeks (9
months).

All exploratory endpoints that were evaluated during the Phase 1/2 study PB-102-F01 will
continue to be assessed in the extension protocol (study PB-102-F02).

The additional exploratory efficacy parameters that were evaluated at baseline of the Phase
1/2 study PB-102-F01 as a reference point, before the initiation of treatment, will be
evaluated after 3 months into the extension study (total treatment of 6 months). These
parameters include Gb3 concentration in renal and skin tissues, LVM, MSSI and cardiac
function tests (echocardiography and stress test). Cerebrovascular disease (clinical and MRI)
will be evaluated at last infusion (total treatment of 12 months).

Inclusion Criteria:

- Completion of Phase 1/2 study PB-102-F01

- The patient signs informed consent

- Female patients and male patients whose co-partners are of child-bearing potential
agree to use a medically acceptable method of contraception, not including the rhythm
method

Exclusion Criteria:

- Pregnant or nursing

- Presence of any medical, emotional, behavioral or psychological condition that, in the
judgment of the Investigator and/or Medical Director, would interfere with the
patient's compliance with the requirements of the study