Affiliation: University of Munich
Beaufort N, Scharrer E, Kremmer E, Lux V, Ehrmann M, Huber R, et al
. Cerebral small vessel disease-related protease HtrA1 processes latent TGF-Î² binding protein 1 and facilitates TGF-Î² signaling. Proc Natl Acad Sci U S A. 2014;111:16496-501 pubmed publisher
..Hence, our data suggest an attenuation of TGF-Î² signaling caused by a lack of HtrA1-mediated LTBP-1 processing as mechanism underlying CARASIL pathogenesis. ..
Haffner C, Vinters H. CADASIL, CARASIL, CARASAL: The linguistic subtleties of cerebral small vessel disease. Neurology. 2016;87:1752-1753 pubmed
Zellner A, Scharrer E, Arzberger T, Oka C, Domenga Denier V, Joutel A, et al
. CADASIL brain vessels show a HTRA1 loss-of-function profile. Acta Neuropathol. 2018;136:111-125 pubmed publisher
..Our study provides evidence for a loss of HTRA1 function as a critical step in the development of CADASIL pathology linking the molecular mechanisms of two distinct SVD forms. ..
Kast J, Hanecker P, Beaufort N, Giese A, Joutel A, Dichgans M, et al
. Sequestration of latent TGF-Î² binding protein 1 into CADASIL-related Notch3-ECD deposits. Acta Neuropathol Commun. 2014;2:96 pubmed publisher
..We propose LTBP-1 as a novel component of Notch3-ECD deposits and suggest its involvement in pathological processes triggered by Notch3-ECD aggregation. ..