Mitochondria abnormalities

Our Appp + hjj jy[ jj]y[ xc jvjo(jei exhibits several aspects of the EBM pathologic phenotype including (a) pronounced vacuolization of most of the muscle fibers (b) congophUicinclusionsinsomeof the muscle fibers (c) aggresome formation (d) nuclear PHFs (e) mitochondria abnormalities including COX deficiency (f) cholesterol accumulation (g) increased (xBC (h) increased parkin (i) increasedmyos-tatin and (j) inability to become innervated [31,141, 214, 221, 230, 231], This cultured human muscle model clearly demonstrates that Appp/A i overexpression can be central to the induction of IBM-characteristic phenotype. 

The mechanism by which the mineral leaves the mitochondrion is only one of the problems of this theory. The mineral in the mitochondrion exists in association with the fluid contents. Thus, unless this water is in some structural form with abnormal solubilities, the mineral must be saturating the fluid, and solubility products apply. It follows that the mitochondrial calcium and phosphate concentrations must be similar to those of the extracellular fluids, i. e. calcium must be concentrated thousandfold to overcome the low intracellular values. 

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