Structural and molecular basis of the assembly of the TRPP2PKD1 complex

• 作 者: Yong Yu Maximilian H.Ulbrich Zafir Buraei Ming-hui Li Xing-Zhen Chen Albert C.M.Ong Liang Tong Ehud Y.Isacoff Jian Yang
• 机 构: DepartmentofBiologicalSciences,ColumbiaUniversity,NewYork,NY10027,USADepartmentofMolecularandCellBio
• 出 处: 2009 harbin conference on lon channels in technology and dru
• 发表日期: 2009年11期

　　Autosomal dominant polycystic kidney disease (ADPKD) is one of the most common genetic diseases in humans.It is caused by mutations in PKD1 and TRPP2.PKD1 (also known as polycystin-1 or PC1) is a 4302-amino acid, 465 kDa integral membrane protein containing 11 putative transmembrane regions.Its large extracellular N terminus contains a number of well recognized repeats and domains, some of which are known to interact with extracellular matrix proteins.The short intracellular C terminus contains a G protein activation site.