This gene encodes a member of the sedolisin family of serine proteases. The protease functions in the lysosome to cleave N-terminal tripeptides from substrates, and has weaker endopeptidase activity. It is synthesized as a catalytically-inactive enzyme which is activated and auto-proteolyzed upon acidification. Mutations in this gene result in late-infantile neuronal ceroid lipofuscinosis, which is associated with the failure to degrade specific neuropeptides and a subunit of ATP synthase in the lysosome. [provided by RefSeq, Jul 2008].
Assani, Yessoufou, Xiong, Segbo, Yu, Zhou, Zhou: "Role of TMPRSS4 Modulation in Breast Cancer Cell Proliferation." in: Asian Pacific journal of cancer prevention : APJCP, Vol. 20, Issue 6, pp. 1849-1856, (2019) (PubMed).
Brenneman, Pearce, Kovacs, DeFrees: "Pharmacological Effects on Ceroid Lipofuscin and Neuronal Structure in Cln3∆ex7/8Mouse Brain Cultures." in: Journal of molecular neuroscience : MN, Vol. 63, Issue 1, pp. 100-114, (2017) (PubMed).
Qing, Zhou, Zhao, Xie, Yang, Xing, Zeng, Jiang: "Altered expression of TPP1 in fibroblast-like synovial cells might be involved in the pathogenesis of rheumatoid arthritis." in: Rheumatology international, Vol. 32, Issue 8, pp. 2503-10, (2012) (PubMed).
Kousi, Siintola, Dvorakova, Vlaskova, Turnbull, Topcu, Yuksel, Gokben, Minassian, Elleder, Mole, Lehesjoki: "Mutations in CLN7/MFSD8 are a common cause of variant late-infantile neuronal ceroid lipofuscinosis." in: Brain : a journal of neurology, Vol. 132, Issue Pt 3, pp. 810-9, (2009) (PubMed).