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MDM4 (MDMX) and its Transcript Variants

MDM family proteins are crucial regulators of the oncosuppressor p53. Alterations of their gene status, mainly amplification events, have been frequently observed in human tumors. MDM4 is one of the two members of the MDM family. The human gene is located on chromosome 1 at q32-33 and codes for a pr...

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Autores principales: Mancini, F, Conza, G. Di, Moretti, F
Formato: Texto
Lenguaje:English
Publicado: Bentham Science Publishers Ltd. 2009
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2699833/
https://www.ncbi.nlm.nih.gov/pubmed/19721810
http://dx.doi.org/10.2174/138920209787581280
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author Mancini, F
Conza, G. Di
Moretti, F
author_facet Mancini, F
Conza, G. Di
Moretti, F
author_sort Mancini, F
collection PubMed
description MDM family proteins are crucial regulators of the oncosuppressor p53. Alterations of their gene status, mainly amplification events, have been frequently observed in human tumors. MDM4 is one of the two members of the MDM family. The human gene is located on chromosome 1 at q32-33 and codes for a protein of 490aa. In analogy to MDM2, besides the full-length mRNA several transcript variants of MDM4 have been identified. Almost all variants thus far described derive from a splicing process, both through canonical and aberrant splicing events. Some of these variants are expressed in normal tissues, others have been observed only in tumor samples. The presence of these variants may be considered a fine tuning of the function of the full-length protein, especially in normal cells. In tumor cells, some variants show oncogenic properties. This review summarizes all the different MDM4 splicing forms thus far described and their role in the regulation of the wild type protein function in normal and tumor cells. In addition, a description of the full-length protein structure with all known interacting proteins thus far identified and a comparison of the MDM4 variant structure with that of full-length protein are presented. Finally, a parallel between MDM4 and MDM2 variants is discussed.
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spelling pubmed-26998332009-09-01 MDM4 (MDMX) and its Transcript Variants Mancini, F Conza, G. Di Moretti, F Curr Genomics Article MDM family proteins are crucial regulators of the oncosuppressor p53. Alterations of their gene status, mainly amplification events, have been frequently observed in human tumors. MDM4 is one of the two members of the MDM family. The human gene is located on chromosome 1 at q32-33 and codes for a protein of 490aa. In analogy to MDM2, besides the full-length mRNA several transcript variants of MDM4 have been identified. Almost all variants thus far described derive from a splicing process, both through canonical and aberrant splicing events. Some of these variants are expressed in normal tissues, others have been observed only in tumor samples. The presence of these variants may be considered a fine tuning of the function of the full-length protein, especially in normal cells. In tumor cells, some variants show oncogenic properties. This review summarizes all the different MDM4 splicing forms thus far described and their role in the regulation of the wild type protein function in normal and tumor cells. In addition, a description of the full-length protein structure with all known interacting proteins thus far identified and a comparison of the MDM4 variant structure with that of full-length protein are presented. Finally, a parallel between MDM4 and MDM2 variants is discussed. Bentham Science Publishers Ltd. 2009-03 /pmc/articles/PMC2699833/ /pubmed/19721810 http://dx.doi.org/10.2174/138920209787581280 Text en ©2009 Bentham Science Publishers Ltd. http://creativecommons.org/licenses/by/2.5/ This is an open access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.5/), which permits unrestrictive use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Article
Mancini, F
Conza, G. Di
Moretti, F
MDM4 (MDMX) and its Transcript Variants
title MDM4 (MDMX) and its Transcript Variants
title_full MDM4 (MDMX) and its Transcript Variants
title_fullStr MDM4 (MDMX) and its Transcript Variants
title_full_unstemmed MDM4 (MDMX) and its Transcript Variants
title_short MDM4 (MDMX) and its Transcript Variants
title_sort mdm4 (mdmx) and its transcript variants
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2699833/
https://www.ncbi.nlm.nih.gov/pubmed/19721810
http://dx.doi.org/10.2174/138920209787581280
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