Inhibition of Intermediate-Conductance Calcium-Activated K Channel (KCa3.1) and Fibroblast Mitogenesis by α-Linolenic Acid and Alterations of Channel Expression in the Lysosomal Storage Disorders, Fabry Disease, and Niemann Pick C.

The comparison of SKA-potentiated and maximal KCa3.1-currents densities in the LSD patients revealed a clear trend toward lower functional KCa3.1 expression in the two hemizygous male FD patients and the male NPC patient but not in heterozygous female FD patients (Figure 5B ): Mean KCa3.1 current densities were reduced to 5 and 20% of control in the two male FD, to 48 and 72% of control in the two female heterozygous FD patients, and to 13% in the male NPC patient.

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