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Structural and Functional Analysis of Disease-Linked p97 ATPase Mutant Complexes

التفاصيل البيبلوغرافية
العنوان: Structural and Functional Analysis of Disease-Linked p97 ATPase Mutant Complexes
المؤلفون: Purbasha Nandi, Shan Li, Rod Carlo A. Columbres, Feng Wang, Dewight R. Williams, Yu-Ping Poh, Tsui-Fen Chou, Po-Lin Chiu
المصدر: International Journal of Molecular Sciences, Vol 22, Iss 15, p 8079 (2021)
بيانات النشر: MDPI AG, 2021.
سنة النشر: 2021
المجموعة: LCC:Biology (General)
LCC:Chemistry
مصطلحات موضوعية: p97 ATPase, p97R155H mutation, p47 cofactor, arginine finger, IBMPFD, single-particle cryo-EM, Biology (General), QH301-705.5, Chemistry, QD1-999
الوصف: IBMPFD/ALS is a genetic disorder caused by a single amino acid mutation on the p97 ATPase, promoting ATPase activity and cofactor dysregulation. The disease mechanism underlying p97 ATPase malfunction remains unclear. To understand how the mutation alters the ATPase regulation, we assembled a full-length p97R155H with its p47 cofactor and first visualized their structures using single-particle cryo-EM. More than one-third of the population was the dodecameric form. Nucleotide presence dissociates the dodecamer into two hexamers for its highly elevated function. The N-domains of the p97R155H mutant all show up configurations in ADP- or ATPγS-bound states. Our functional and structural analyses showed that the p47 binding is likely to impact the p97R155H ATPase activities via changing the conformations of arginine fingers. These functional and structural analyses underline the ATPase dysregulation with the miscommunication between the functional modules of the p97R155H.
نوع الوثيقة: article
وصف الملف: electronic resource
اللغة: English
تدمد: 1422-0067
1661-6596
Relation: https://www.mdpi.com/1422-0067/22/15/8079; https://doaj.org/toc/1661-6596; https://doaj.org/toc/1422-0067
DOI: 10.3390/ijms22158079
URL الوصول: https://doaj.org/article/9f08726992584fa1ba2a51f08262e895
رقم الأكسشن: edsdoj.9f08726992584fa1ba2a51f08262e895
قاعدة البيانات: Directory of Open Access Journals
الوصف
تدمد:14220067
16616596
DOI:10.3390/ijms22158079