Structural analysis of the full-length human LRRK2

التفاصيل البيبلوغرافية
العنوان: Structural analysis of the full-length human LRRK2
المؤلفون: Alexander G. Myasnikov, Junmin Peng, Boer Xie, Patricia Hixson, Kaiwen Yu, Aaron Pitre, Ji Sun, Hanwen Zhu
المصدر: Cell
سنة النشر: 2021
مصطلحات موضوعية: Models, Molecular, Biology, medicine.disease_cause, Leucine-Rich Repeat Serine-Threonine Protein Kinase-2, General Biochemistry, Genetics and Molecular Biology, Protein Structure, Secondary, Article, Pathogenesis, 03 medical and health sciences, Membranous organelles, 0302 clinical medicine, Protein Domains, Microtubule, medicine, Humans, Amino Acid Sequence, 030304 developmental biology, 0303 health sciences, Mutation, Kinase, LRRK2, Cell biology, nervous system diseases, HEK293 Cells, Protein kinase domain, Protein Multimerization, 030217 neurology & neurosurgery
الوصف: Mutations in leucine-rich repeat kinase 2 (LRRK2) are commonly implicated in the pathogenesis of both familial and sporadic Parkinson’s disease (PD). LRRK2 regulates critical cellular processes at membranous organelles and forms microtubule-based pathogenic filaments, yet the molecular basis underlying these biological roles of LRRK2 remains largely enigmatic. Here we determined high-resolution structures of full-length human LRRK2, revealing its architecture and key interdomain scaffolding elements for rationalizing disease-causing mutations. The kinase domain of LRRK2 is captured in an inactive state, a conformation also adopted by the most common PD-associated mutation, LRRK2(G2019S). This conformation serves as a framework for structure-guided design of conformational specific inhibitors. We further determined the structure of COR-mediated LRRK2 dimers and found that single-point mutations at the dimer interface abolished pathogenic filamentation in cells. Overall, our study provides mechanistic insights into physiological and pathological roles of LRRK2 and establishes a structural template for future therapeutic intervention in PD.
تدمد: 1097-4172
URL الوصول: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::f889752ec4ac6ce5aa9fef896d5e833f
https://pubmed.ncbi.nlm.nih.gov/34107286
Rights: OPEN
رقم الانضمام: edsair.doi.dedup.....f889752ec4ac6ce5aa9fef896d5e833f
قاعدة البيانات: OpenAIRE