Differential roles of PML isoforms

The tumor suppressor promyelocytic leukemia protein (PML) is fused to the retinoic acid receptor alpha in patients suffering from acute promyelocytic leukemia (APL). Treatment of APL patients with arsenic trioxide (As2O3) reverses the disease phenotype by a process involving the degradation of the f...

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Main Authors: Sébastien eNisole, Mohamed Ali eMaroui, Xavier eMascle, Muriel eAubry, Mounira K Chelbi-Alix
Format: Article
Language:English
Published: Frontiers Media S.A. 2013-05-01
Series:Frontiers in Oncology
Subjects:
Online Access:http://journal.frontiersin.org/Journal/10.3389/fonc.2013.00125/full
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author Sébastien eNisole
Sébastien eNisole
Mohamed Ali eMaroui
Mohamed Ali eMaroui
Xavier eMascle
Muriel eAubry
Mounira K Chelbi-Alix
Mounira K Chelbi-Alix
author_facet Sébastien eNisole
Sébastien eNisole
Mohamed Ali eMaroui
Mohamed Ali eMaroui
Xavier eMascle
Muriel eAubry
Mounira K Chelbi-Alix
Mounira K Chelbi-Alix
author_sort Sébastien eNisole
collection DOAJ
description The tumor suppressor promyelocytic leukemia protein (PML) is fused to the retinoic acid receptor alpha in patients suffering from acute promyelocytic leukemia (APL). Treatment of APL patients with arsenic trioxide (As2O3) reverses the disease phenotype by a process involving the degradation of the fusion protein via its PML moiety. Several PML isoforms are generated from a single PML gene by alternative splicing. They share the same N-terminal region containing the RBCC/TRIM motif but differ in their C-terminal sequences. Recent studies of all the PML isoforms reveal the specific functions of each. Here, we review the nomenclature and structural organization of the PML isoforms in order to clarify the various designations and classifications found in different databases. The functions of the PML isoforms and their differential roles in antiviral defense also are reviewed. Finally, the key players involved in the degradation of the PML isoforms in response to As2O3 or other inducers are discussed.
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spelling doaj.art-e60ed5abc7bf4ecea9578a50435858ac2022-12-21T23:57:36ZengFrontiers Media S.A.Frontiers in Oncology2234-943X2013-05-01310.3389/fonc.2013.0012551002Differential roles of PML isoformsSébastien eNisole0Sébastien eNisole1Mohamed Ali eMaroui2Mohamed Ali eMaroui3Xavier eMascle4Muriel eAubry5Mounira K Chelbi-Alix6Mounira K Chelbi-Alix7INSERM UMR-S 747Université Paris DescartesCNRS FRE3235Université Paris DescartesUniversité de MontréalUniversité de MontréalCNRS FRE3235Université Paris DescartesThe tumor suppressor promyelocytic leukemia protein (PML) is fused to the retinoic acid receptor alpha in patients suffering from acute promyelocytic leukemia (APL). Treatment of APL patients with arsenic trioxide (As2O3) reverses the disease phenotype by a process involving the degradation of the fusion protein via its PML moiety. Several PML isoforms are generated from a single PML gene by alternative splicing. They share the same N-terminal region containing the RBCC/TRIM motif but differ in their C-terminal sequences. Recent studies of all the PML isoforms reveal the specific functions of each. Here, we review the nomenclature and structural organization of the PML isoforms in order to clarify the various designations and classifications found in different databases. The functions of the PML isoforms and their differential roles in antiviral defense also are reviewed. Finally, the key players involved in the degradation of the PML isoforms in response to As2O3 or other inducers are discussed.http://journal.frontiersin.org/Journal/10.3389/fonc.2013.00125/fullvirusSUMORNF4CK2TRIMPML isoforms
spellingShingle Sébastien eNisole
Sébastien eNisole
Mohamed Ali eMaroui
Mohamed Ali eMaroui
Xavier eMascle
Muriel eAubry
Mounira K Chelbi-Alix
Mounira K Chelbi-Alix
Differential roles of PML isoforms
Frontiers in Oncology
virus
SUMO
RNF4
CK2
TRIM
PML isoforms
title Differential roles of PML isoforms
title_full Differential roles of PML isoforms
title_fullStr Differential roles of PML isoforms
title_full_unstemmed Differential roles of PML isoforms
title_short Differential roles of PML isoforms
title_sort differential roles of pml isoforms
topic virus
SUMO
RNF4
CK2
TRIM
PML isoforms
url http://journal.frontiersin.org/Journal/10.3389/fonc.2013.00125/full
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AT xavieremascle differentialrolesofpmlisoforms
AT murieleaubry differentialrolesofpmlisoforms
AT mounirakchelbialix differentialrolesofpmlisoforms
AT mounirakchelbialix differentialrolesofpmlisoforms