Immuno-proteasome

In S-IBM muscle fibers, our studies have demonstrated proteasomal abnormalities, as evidenced by (a) abnormal accumulation of 26S proteasome subunits by immunocytochemistry and immuno-electron microscopy (b) increased expression of 26S proteasome subunits by immunoblots but, contrastingly (c) reduced activities of the three major proteasomal proteolytic enzymes [11]. This indicates accumulation of hypo-active/inactive proteasomal subunit proteins. 

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