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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).

References associated with 9.B.192 family:

Egawa, K., H. Shibata, S. Yamashita, H. Yurimoto, Y. Sakai, and H. Kato. (2009). Overexpression and purification of rat peroxisomal membrane protein 22, PMP22, in Pichia pastoris. Protein Expr Purif 64: 47-54. 18984054
Karadimas, C.L., T.H. Vu, S.A. Holve, P. Chronopoulou, C. Quinzii, S.D. Johnsen, J. Kurth, E. Eggers, L. Palenzuela, K. Tanji, E. Bonilla, D.C. De Vivo, S. DiMauro, and M. Hirano. (2006). Navajo neurohepatopathy is caused by a mutation in the MPV17 gene. Am J Hum Genet 79: 544-548. 16909392
Magyar, J.P., C. Ebensperger, N. Schaeren-Wiemers, and U. Suter. (1997). Myelin and lymphocyte protein (MAL/MVP17/VIP17) and plasmolipin are members of an extended gene family. Gene 189: 269-275. 9168137
Simske, J.S. (2013). Claudins reign: The claudin/EMP/PMP22/γ channel protein family in C. elegans. Tissue Barriers 1: e25502. 24665403
Trott, A. and K.A. Morano. (2004). SYM1 is the stress-induced Saccharomyces cerevisiae ortholog of the mammalian kidney disease gene Mpv17 and is required for ethanol metabolism and tolerance during heat shock. Eukaryot. Cell. 3: 620-631. 15189984