Role of Wiskott Aldrich syndrome protein in haematological malignancies: genetics, molecular mechanisms and therapeutic strategies.

Autor: R P; Department of Biotechnology, SRM Institute of Science and Technology, Katangulathur, Tamil Nadu 603203, India., Shanmugam G; Department of Biotechnology, SRM Institute of Science and Technology, Katangulathur, Tamil Nadu 603203, India., Rakshit S; Department of Biotechnology, SRM Institute of Science and Technology, Katangulathur, Tamil Nadu 603203, India., Sarkar K; Department of Biotechnology, SRM Institute of Science and Technology, Katangulathur, Tamil Nadu 603203, India. Electronic address: koustavsarkar@gmail.com.
Jazyk: angličtina
Zdroj: Pathology, research and practice [Pathol Res Pract] 2024 Jan; Vol. 253, pp. 155026. Date of Electronic Publication: 2023 Dec 10.
DOI: 10.1016/j.prp.2023.155026
Abstrakt: As patients continue to suffer from lymphoproliferative and myeloproliferative diseases known as haematopoietic malignancies can affect the bone marrow, blood, lymph nodes, and lymphatic and non-lymphatic organs. Despite advances in the current treatment, there is still a significant challenge for physicians to improve the therapy of HMs. WASp is an important regulator of actin polymerization and the involvement of WASp in transcription is thought to be linked to the DNA damage response and repair. In some studies, severe immunodeficiency and lymphoid malignancy are caused by WASp mutations or the absence of WASp and these mutations in WAS can alter the function and/or expression of the intracellular protein. Loss-of-function and Gain-of-function mutations in WASp have an impact on cancer malignancies' incidence and onset. Recent studies suggest that depending on the clinical or experimental situation, WASPs and WAVEs can operate as a suppressor or enhancers for cancer malignancy. These dual functions of WASPs and WAVEs in cancer likely arose from their multifaceted role in cells that could be targeted for anticancer drug development. The significant role and their association of WASp in Chronic myeloid leukaemia, Juvenile myelomonocytic leukaemia and T-cell lymphoma is discussed. In this review, we described the structure and function of WASp and its family mechanism, analysing major regulatory effectors and summarising the clinical relevance and drugs that specifically target WASp in disease treatment in various hematopoietic malignancies by different approaches.
Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.
(Copyright © 2023 Elsevier GmbH. All rights reserved.)
Databáze: MEDLINE
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