9.B.192 The Mpv17/Pmp22 4 TMS Putative Porin (Mpv17) Family
The 22-kDa peroxisomal membrane protein (PMP22) is a component of peroxisomal membranes. PMP22 seems to be involved in pore forming activity and may contribute to the unspecific permeability of the organelle membrane (Simske 2013). PMP22 is synthesized on free cytosolic ribosomes and then directed to the peroxisome membrane by specific targeting information, although this is not universally accepted (Egawa et al. 2009). Mpv17 is a closely related peroxisomal protein. In mouse, the Mpv17 protein is involved in the development of early-onset glomerulosclerosis. A homolog of Mpv17 in S. cerevisiae has been been found to be an integral membrane protein of the inner mitochondrial membrane where it has been proposed to have a role in ethanol metabolizm and tolerance during heat-shock (Trott and Morano 2004). Defects in MPV17 are associated with mitochondrial DNA depletion syndrome (MDDS) and Navajo neurohepatopathy (NNH) (Karadimas et al. 2006).