TYSND1 cleaves peroxisomal proteins

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R-HSA-9033500
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Homo sapiens
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After proteins are imported into the peroxisome a subset of proteins are cleaved by the protease TYSND1 (Okumoto et al. 2011). Based onmutagenesis of human TYSND1 (Okumoto et al. 2011) and the homolog in Arabidopsis (Schuhmann et al. 2008), TYSND1 appears to be a trypsin-like serine protease containing a conserved histidine aspartate serine triad essential for catalysis. Mice lacking Tysnd1 have reduced peroxisomal localization of some peroxisomal enzymes and exhibit reduced beta-oxidation of fatty acids and metabolism of phytanic acid (Mizuno et al. 2013). Male mice lacking Tysnd1 are sterile due to sperm that lack acrosomal caps.

Literature References
PubMed ID Title Journal Year
23459139 Tysnd1 deficiency in mice interferes with the peroxisomal localization of PTS2 enzymes, causing lipid metabolic abnormalities and male infertility

Mizuno, Y, Ninomiya, Y, Nakachi, Y, Iseki, M, Iwasa, H, Akita, M, Tsukui, T, Shimozawa, N, Ito, C, Toshimori, K, Nishimukai, M, Hara, H, Maeba, R, Okazaki, T, Alodaib, AN, Al Amoudi, M, Jacob, M, Alkuraya, FS, Horai, Y, Watanabe, M, Motegi, H, Wakana, S, Noda, T, Kurochkin, IV, Mizuno, Y, Sch├Ânbach, C, Okazaki, Y

PLoS Genet. 2013
22002062 Two proteases, trypsin domain-containing 1 (Tysnd1) and peroxisomal lon protease (PsLon), cooperatively regulate fatty acid ╬▓-oxidation in peroxisomal matrix

Okumoto, K, Kametani, Y, Fujiki, Y

J. Biol. Chem. 2011
18952862 The DEG15 serine protease cleaves peroxisomal targeting signal 2-containing proteins in Arabidopsis

Schuhmann, H, Huesgen, PF, Gietl, C, Adamska, I

Plant Physiol. 2008
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