M. Kool

Mapping pediatric brain tumors to their origins in the developing cerebellum

The paper maps the cellular origins of three common pediatric brain tumors using a single-nucleus atlas and transcriptome data, identifying lineage-specific origins and potential therapeutic targets.

Recurrent fusions in PLAGL1 define a distinct subset of pediatric-type supratentorial ependymoma

The paper identifies a novel subset of supratentorial ependymomas in children characterized by recurrent PLAGL1 fusions and distinct molecular features.

A synergistic interaction between HDAC- and PARP inhibitors in childhood tumors with chromothripsis

The paper identifies a synergistic effect between HDAC and PARP inhibitors in childhood tumors with chromothripsis, validated through drug screening and in vivo models.

Osteoprotegerin is elevated in pulmonary fibrosis and associates with IPF progression

This study shows OPG levels are elevated in fibrotic lung tissue and correlate with IPF disease progression and reduced lung function.

PPM1D is a neuroblastoma oncogene and therapeutic target in childhood neural tumors.

This study identifies PPM1D as an oncogene and therapeutic target in neuroblastoma by showing its role in p53 inactivation and tumor growth.

SMARCB1 loss interacts with neuronal differentiation state to block maturation and impact cell stability

This study shows SMARCB1 loss affects neuronal differentiation and cell stability differently based on the differentiation state, contributing to ATRT tumorigenesis.

Cellular mRNA signals in human kidney tumors

This study analyzes mRNA signals in 1,300 kidney tumors to determine their differentiation state and origin, finding that childhood tumors show fetal signals while most adult tumors do not, with exceptions in certain cancer types.

Functional loss of a non-canonical BCOR-PRC1.1 complex accelerates SHH-driven medulloblastoma formation

This study shows that loss of BCOR in SHH-medulloblastoma accelerates tumor formation by disrupting the BCOR-PRC1.1 complex and increasing Igf2 expression.

Defective DNA damage repair leads to frequent catastrophic genomic events in murine and human tumors

The paper identifies chromothripsis and chromoanasynthesis in tumors with DNA repair deficiencies and links them to MYC/MYCN amplification and therapeutic opportunities.