Figure 4.

Western blot analysis in patients with Danon disease. (A,B) Skeletal muscle extracts from a male control and a female control, and the male patient and female patient, who were blotted and labeled with antibodies against LAMP-2 and β–actin. (A) The male patient and female patient from Family 5; (B) the male patient from Family 5 and the female patient from Family 13, reconstructed with permission from our previous reports [8,9]. (C) The relative protein expression levels in the male controls were expressed as 100%. LAMP-2 immunoreactivity was present equally in the both male and female control muscles. In contrast, LAMP-2 was undetectable in the male patients’ muscle, whereas two female patients showed decreased LAMP-2.