Functional and epigenetic phenotypes of humans and mice with DNMT3A Overgrowth Syndrome

AM Smith, TA LaValle, M Shinawi… - Nature …, 2021 - nature.com
Germline pathogenic variants in DNMT3A were recently described in patients with
overgrowth, obesity, behavioral, and learning difficulties (D NMT3A O vergrowth S …

Mutations in the DNA methyltransferase gene DNMT3A cause an overgrowth syndrome with intellectual disability

K Tatton-Brown, S Seal, E Ruark, J Harmer… - Nature …, 2014 - nature.com
Overgrowth disorders are a heterogeneous group of conditions characterized by increased
growth parameters and other variable clinical features such as intellectual disability and …

DNMT3A overgrowth syndrome is associated with the development of hematopoietic malignancies in children and young adults

MA Ferris, AM Smith, SE Heath… - Blood, The Journal …, 2022 - ashpublications.org
DNMT3A Overgrowth Syndrome (DOS, also known as Tatton-Brown Rahman
Syndrome/TBRS) is one of several overgrowth syndromes with complex phenotypes caused …

Dnmt3a loss predisposes murine hematopoietic stem cells to malignant transformation

A Mayle, L Yang, B Rodriguez, T Zhou… - Blood, The Journal …, 2015 - ashpublications.org
Abstract DNA methyltransferase 3A (DNMT3A) is mutated in hematologic malignancies
affecting myeloid, mixed, and lymphoid lineages, and these mutations are associated with …

Acute myeloid leukemia‐associated DNMT3A p.Arg882His mutation in a patient with Tatton‐Brown–Rahman overgrowth syndrome as a constitutional mutation

R Kosaki, H Terashima, M Kubota… - American Journal of …, 2017 - Wiley Online Library
DNA methylation plays a critical role in both embryonic development and tumorigenesis and
is mediated through various DNA methyltransferases. Constitutional mutations in the de …

Loss of Dnmt3a immortalizes hematopoietic stem cells in vivo

M Jeong, HJ Park, H Celik, EL Ostrander, JM Reyes… - Cell reports, 2018 - cell.com
Somatic mutations in DNMT3A are recurrent events across a range of blood cancers.
Dnmt3a loss of function in hematopoietic stem cells (HSCs) skews divisions toward self …

DNMT3A mutations in acute myeloid leukemia

MY Shah, JD Licht - Nature genetics, 2011 - nature.com
New studies reveal that 20% of individuals with acute myeloid leukemia harbor somatic
mutations in DNMT3A (encoding DNA methyltransferase 3A). Although these leukemias …

A Model System for Studying the DNMT3A Hotspot Mutation (DNMT3AR882) Demonstrates a Causal Relationship between Its Dominant-Negative Effect and …

R Lu, J Wang, Z Ren, J Yin, Y Wang, L Cai, GG Wang - Cancer research, 2019 - AACR
Mutation of DNA methyltransferase 3A at arginine 882 (DNMT3AR882mut) is prevalent in
hematologic cancers and disorders. Recently, DNMT3AR882mut has been shown to have …

Recurrent DNMT3A mutations in patients with myelodysplastic syndromes

MJ Walter, L Ding, D Shen, J Shao, M Grillot… - Leukemia, 2011 - nature.com
Alterations in DNA methylation have been implicated in the pathogenesis of myelodysplastic
syndromes (MDS), although the underlying mechanism remains largely unknown …

DNMT3A haploinsufficiency causes dichotomous DNA methylation defects at enhancers in mature human immune cells

JY Lim, SH Duttke, TS Baker, J Lee… - Journal of Experimental …, 2021 - rupress.org
DNMT3A encodes an enzyme that carries out de novo DNA methylation, which is essential
for the acquisition of cellular identity and specialized functions during cellular differentiation …