8GBC

Homo sapiens Zalpha mutant - N173S

Summary for 8GBC

• Entry DOI: 10.2210/pdb8gbc/pdb
• NMR Information: BMRB: 51833
• Descriptor: Double-stranded RNA-specific adenosine deaminase (1 entity in total)
• Functional Keywords: rna binding protein
• Biological source: Homo sapiens (human)
• Total number of polymer chains: 1
• Total formula weight: 9234.65

Authors

Langeberg, C.J.,Nichols, P.J.,Henen, M.,Vicens, Q.,Vogeli, B. (deposition date: 2023-02-25, release date: 2023-03-22, Last modification date: 2024-05-15)

Primary citation

Langeberg, C.J.,Nichols, P.J.,Henen, M.A.,Vicens, Q.,Vogeli, B.
Differential Structural Features of Two Mutant ADAR1p150 Z alpha Domains Associated with Aicardi-Goutieres Syndrome.
J.Mol.Biol., 435:168040-168040, 2023

PubMed Abstract: The Zα domain of ADARp150 is critical for proper Z-RNA substrate binding and is a key factor in the type-I interferon response pathway. Two point-mutations in this domain (N173S and P193A), which cause neurodegenerative disorders, are linked to decreased A-to-I editing in disease models. To understand this phenomenon at the molecular level, we biophysically and structurally characterized these two mutated domains, revealing that they bind Z-RNA with a decreased affinity. Less efficient binding to Z-RNA can be explained by structural changes in beta-wing, part of the Z-RNA-protein interface, and alteration of conformational dynamics of the proteins.

PubMed: 36889460
DOI: 10.1016/j.jmb.2023.168040

Experimental method

SOLUTION NMR