Conformational consequences of NPM1 rare mutations: An aggregation perspective in Acute Myeloid Leukemia

Bioorg Chem. 2021 Aug:113:104997. doi: 10.1016/j.bioorg.2021.104997. Epub 2021 May 18.

Abstract

Often proteins association is a physiological process used by cells to regulate their growth and to adapt to different stress conditions, including mutations. In the case of a subtype of Acute Myeloid Leukemia (AML), mutations of nucleophosmin 1 (NPM1) protein cause its aberrant cytoplasmatic mislocalization (NPMc+). We recently pointed out an amyloidogenic propensity of protein regions including the most common mutations of NPMc+ located in the C-terminal domain (CTD): they were able to form, in vitro, amyloid cytotoxic aggregates with fibrillar morphology. Herein, we analyzed the conformational characteristics of several peptides including rare AML mutations of NPMc+. By means of different spectroscopic, microscopic and cellular assays we evaluated the importance of amino acid composition, among rare AML mutations, to determine amyloidogenic propensity. This study could add a piece of knowledge to the structural consequences of mutations in cytoplasmatic NPM1c+.

Keywords: Acute Myeloid Leukemia; Aggregation; Amyloid fibers; NPM1 mutations.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Humans
  • Leukemia, Myeloid, Acute / genetics*
  • Leukemia, Myeloid, Acute / metabolism
  • Mutation
  • Nuclear Proteins / analysis
  • Nuclear Proteins / genetics*
  • Nuclear Proteins / metabolism
  • Nucleophosmin
  • Protein Aggregates
  • Protein Conformation
  • Tumor Cells, Cultured

Substances

  • NPM1 protein, human
  • Nuclear Proteins
  • Protein Aggregates
  • Nucleophosmin