KMT2A degradation is observed in decitabine‐responsive acute lymphoblastic leukemia cells

Hypermethylation of tumor suppressor genes is a hallmark of leukemia. The hypomethylating agent decitabine covalently binds, and degrades DNA (cytosine‐5)‐methyltransferase 1 (DNMT1). Structural similarities within DNA‐binding domains of DNMT1, and the leukemic driver histone‐lysine N‐methyltransfer...

Full description

Saved in:
Bibliographic Details
Main Authors: Luisa Brock, Lina Benzien, Sandra Lange, Maja Huehns, Alexandra Runge, Catrin Roolf, Anett Sekora, Gudrun Knuebel, Hugo Murua Escobar, Christian Junghanss, Anna Richter
Format: Article
Language:English
Published: Wiley 2025-05-01
Series:Molecular Oncology
Subjects:
Online Access:https://doi.org/10.1002/1878-0261.13792
Tags: Add Tag
No Tags, Be the first to tag this record!
_version_ 1850031640282136576
author Luisa Brock
Lina Benzien
Sandra Lange
Maja Huehns
Alexandra Runge
Catrin Roolf
Anett Sekora
Gudrun Knuebel
Hugo Murua Escobar
Christian Junghanss
Anna Richter
author_facet Luisa Brock
Lina Benzien
Sandra Lange
Maja Huehns
Alexandra Runge
Catrin Roolf
Anett Sekora
Gudrun Knuebel
Hugo Murua Escobar
Christian Junghanss
Anna Richter
author_sort Luisa Brock
collection DOAJ
description Hypermethylation of tumor suppressor genes is a hallmark of leukemia. The hypomethylating agent decitabine covalently binds, and degrades DNA (cytosine‐5)‐methyltransferase 1 (DNMT1). Structural similarities within DNA‐binding domains of DNMT1, and the leukemic driver histone‐lysine N‐methyltransferase 2A (KMT2A) suggest that decitabine might also affect the latter. In acute lymphoblastic leukemia (ALL) cell lines, and xenograft models, we observed increased DNMT1, and KMT2A expression in response to decitabine‐induced demethylation. Strikingly, KMT2A protein expression was diminished in all cell lines that experienced DNMT1 degradation. Moreover, only cells with reduced KMT2A protein levels showed biological effects following decitabine treatment. KMT2A wild‐type, and rearranged cells were locked in G2 and G1 cell cycle phases, respectively, likely due to p27/p16 activation. Primary sample gene expression profiling confirmed different patterns between KMT2A wild‐type, and translocated cells. This newly discovered decitabine mode of action via KMT2A degradation evokes anti‐leukemic activity in adult ALL cells, and can act synergistically with menin inhibition. Following the successful clinical implementation of decitabine for acute myeloid leukemia, the drug should be considered a potential promising addition to the therapeutic portfolio for ALL as well.
format Article
id doaj-art-97bddb984e144def8111acc976c342e4
institution DOAJ
issn 1574-7891
1878-0261
language English
publishDate 2025-05-01
publisher Wiley
record_format Article
series Molecular Oncology
spelling doaj-art-97bddb984e144def8111acc976c342e42025-08-20T02:58:55ZengWileyMolecular Oncology1574-78911878-02612025-05-011951404142110.1002/1878-0261.13792KMT2A degradation is observed in decitabine‐responsive acute lymphoblastic leukemia cellsLuisa Brock0Lina Benzien1Sandra Lange2Maja Huehns3Alexandra Runge4Catrin Roolf5Anett Sekora6Gudrun Knuebel7Hugo Murua Escobar8Christian Junghanss9Anna Richter10Department of Medicine, Clinic III – Hematology, Oncology, Palliative Medicine Rostock University Medical Center GermanyDepartment of Medicine, Clinic III – Hematology, Oncology, Palliative Medicine Rostock University Medical Center GermanyDepartment of Medicine, Clinic III – Hematology, Oncology, Palliative Medicine Rostock University Medical Center GermanyInstitute of Pathology Rostock University Medical Center GermanyDepartment of Medicine, Clinic III – Hematology, Oncology, Palliative Medicine Rostock University Medical Center GermanyDepartment of Medicine, Clinic III – Hematology, Oncology, Palliative Medicine Rostock University Medical Center GermanyDepartment of Medicine, Clinic III – Hematology, Oncology, Palliative Medicine Rostock University Medical Center GermanyDepartment of Medicine, Clinic III – Hematology, Oncology, Palliative Medicine Rostock University Medical Center GermanyDepartment of Medicine, Clinic III – Hematology, Oncology, Palliative Medicine Rostock University Medical Center GermanyDepartment of Medicine, Clinic III – Hematology, Oncology, Palliative Medicine Rostock University Medical Center GermanyDepartment of Medicine, Clinic III – Hematology, Oncology, Palliative Medicine Rostock University Medical Center GermanyHypermethylation of tumor suppressor genes is a hallmark of leukemia. The hypomethylating agent decitabine covalently binds, and degrades DNA (cytosine‐5)‐methyltransferase 1 (DNMT1). Structural similarities within DNA‐binding domains of DNMT1, and the leukemic driver histone‐lysine N‐methyltransferase 2A (KMT2A) suggest that decitabine might also affect the latter. In acute lymphoblastic leukemia (ALL) cell lines, and xenograft models, we observed increased DNMT1, and KMT2A expression in response to decitabine‐induced demethylation. Strikingly, KMT2A protein expression was diminished in all cell lines that experienced DNMT1 degradation. Moreover, only cells with reduced KMT2A protein levels showed biological effects following decitabine treatment. KMT2A wild‐type, and rearranged cells were locked in G2 and G1 cell cycle phases, respectively, likely due to p27/p16 activation. Primary sample gene expression profiling confirmed different patterns between KMT2A wild‐type, and translocated cells. This newly discovered decitabine mode of action via KMT2A degradation evokes anti‐leukemic activity in adult ALL cells, and can act synergistically with menin inhibition. Following the successful clinical implementation of decitabine for acute myeloid leukemia, the drug should be considered a potential promising addition to the therapeutic portfolio for ALL as well.https://doi.org/10.1002/1878-0261.13792acute leukemiadecitabineDNMT1KMT2Ameninrevumenib
spellingShingle Luisa Brock
Lina Benzien
Sandra Lange
Maja Huehns
Alexandra Runge
Catrin Roolf
Anett Sekora
Gudrun Knuebel
Hugo Murua Escobar
Christian Junghanss
Anna Richter
KMT2A degradation is observed in decitabine‐responsive acute lymphoblastic leukemia cells
Molecular Oncology
acute leukemia
decitabine
DNMT1
KMT2A
menin
revumenib
title KMT2A degradation is observed in decitabine‐responsive acute lymphoblastic leukemia cells
title_full KMT2A degradation is observed in decitabine‐responsive acute lymphoblastic leukemia cells
title_fullStr KMT2A degradation is observed in decitabine‐responsive acute lymphoblastic leukemia cells
title_full_unstemmed KMT2A degradation is observed in decitabine‐responsive acute lymphoblastic leukemia cells
title_short KMT2A degradation is observed in decitabine‐responsive acute lymphoblastic leukemia cells
title_sort kmt2a degradation is observed in decitabine responsive acute lymphoblastic leukemia cells
topic acute leukemia
decitabine
DNMT1
KMT2A
menin
revumenib
url https://doi.org/10.1002/1878-0261.13792
work_keys_str_mv AT luisabrock kmt2adegradationisobservedindecitabineresponsiveacutelymphoblasticleukemiacells
AT linabenzien kmt2adegradationisobservedindecitabineresponsiveacutelymphoblasticleukemiacells
AT sandralange kmt2adegradationisobservedindecitabineresponsiveacutelymphoblasticleukemiacells
AT majahuehns kmt2adegradationisobservedindecitabineresponsiveacutelymphoblasticleukemiacells
AT alexandrarunge kmt2adegradationisobservedindecitabineresponsiveacutelymphoblasticleukemiacells
AT catrinroolf kmt2adegradationisobservedindecitabineresponsiveacutelymphoblasticleukemiacells
AT anettsekora kmt2adegradationisobservedindecitabineresponsiveacutelymphoblasticleukemiacells
AT gudrunknuebel kmt2adegradationisobservedindecitabineresponsiveacutelymphoblasticleukemiacells
AT hugomuruaescobar kmt2adegradationisobservedindecitabineresponsiveacutelymphoblasticleukemiacells
AT christianjunghanss kmt2adegradationisobservedindecitabineresponsiveacutelymphoblasticleukemiacells
AT annarichter kmt2adegradationisobservedindecitabineresponsiveacutelymphoblasticleukemiacells