Oligomerization, trans-reduction, and instability of mutant NOTCH3 in inherited vascular dementia

Kelly Z. Young; Carolina Rojas Ramírez; Simon G. Keep; John R. Gatti; Soo Jung Lee; Xiaojie Zhang; Magdalena I. Ivanova; Brandon T. Ruotolo; Michael M. Wang

doi:10.1038/s42003-022-03259-2

Communications Biology (Apr 2022)

Oligomerization, trans-reduction, and instability of mutant NOTCH3 in inherited vascular dementia

Kelly Z. Young,
Carolina Rojas Ramírez,
Simon G. Keep,
John R. Gatti,
Soo Jung Lee,
Xiaojie Zhang,
Magdalena I. Ivanova,
Brandon T. Ruotolo,
Michael M. Wang

Affiliations

Kelly Z. Young: Departments of Neurology, University of Michigan
Carolina Rojas Ramírez: Department of Chemistry, University of Michigan
Simon G. Keep: Departments of Neurology, University of Michigan
John R. Gatti: The Johns Hopkins School of Medicine
Soo Jung Lee: Departments of Neurology, University of Michigan
Xiaojie Zhang: Departments of Neurology, University of Michigan
Magdalena I. Ivanova: Departments of Neurology, University of Michigan
Brandon T. Ruotolo: Department of Chemistry, University of Michigan
Michael M. Wang: Departments of Neurology, University of Michigan

DOI: https://doi.org/10.1038/s42003-022-03259-2
Journal volume & issue: Vol. 5, no. 1
pp. 1 – 12

Abstract

Read online

Specific cysteine residue alterations and quaternary structures are induced by CADASIL mutations in NOTCH3, which are found to induce oligomeric states, altered disulphide bonding, increased free thiols and reduced protein stability.

Published in Communications Biology

ISSN: 2399-3642 (Online)
Publisher: Nature Portfolio
Country of publisher: United Kingdom
LCC subjects: Science: Biology (General)
Website: https://www.nature.com/commsbio/

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