p27<sup>Kip1</sup> and Tumors: Characterization of <i>CDKN1B</i> Variants Identified in MEN4 and Breast Cancer

p27<sup>Kip1</sup> is a key cell cycle gatekeeper governing the timing of Cyclin-dependent kinase (CDK) activation/inactivation and, consequently, cell proliferation. Structurally, the protein is largely unfolded, a feature that strongly increases its plasticity and interactors and enhan...

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Main Authors: Debora Bencivenga, Emanuela Stampone, Jahanzaib Azhar, Daniela Parente, Waqar Ali, Vitale Del Vecchio, Fulvio Della Ragione, Adriana Borriello
Format: Article
Language:English
Published: MDPI AG 2025-01-01
Series:Cells
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Online Access:https://www.mdpi.com/2073-4409/14/3/188
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Summary:p27<sup>Kip1</sup> is a key cell cycle gatekeeper governing the timing of Cyclin-dependent kinase (CDK) activation/inactivation and, consequently, cell proliferation. Structurally, the protein is largely unfolded, a feature that strongly increases its plasticity and interactors and enhances the number of regulated cellular processes. p27<sup>Kip1</sup>, like other intrinsically unstructured proteins, is post-translationally modified on several residues. These modifications affect its cellular localization and address p27<sup>Kip1</sup> for specific interactions/functions. Several germline or somatic <i>CDKN1B</i> (the p27<sup>Kip1</sup> encoding gene) mutations have been demonstrated to be associated with multiple endocrine neoplasia type 4 (MEN4), hairy cell leukemia, small-intestine neuroendocrine tumors, and breast and prostate cancers. Here, we analyzed the effect of four <i>CDKN1B</i> missense and nonsense mutations found in patients affected by MEN4 or cancers, namely, c.349C>T, p.P117S; c.397C>A, p.P133T; c.487C>T, p.Q163*; and c.511G>T, p.E171*. By transfecting breast cancer cell lines, we observed increased growth and cell motility for all the investigated mutants compared to wild-type p27<sup>Kip1</sup> transfected cells. Furthermore, we discovered that the mutant forms exhibited altered phosphorylation on key residues and different localization or degradation mechanisms in comparison to the wild-type protein and suggested a possible region as crucial for the lysosome-dependent degradation of the protein. Finally, the loss of p27<sup>Kip1</sup> ability in blocking cell proliferation was in part explained through the different binding efficiency that mutant p27<sup>Kip1</sup> forms exhibited with Cyclin/Cyclin-dependent Kinase complexes (or proteins involved indirectly in that binding) with respect to the WT.
ISSN:2073-4409