The role of p53 in developmental syndromes

ME Bowen, LD Attardi - Journal of molecular cell biology, 2019 - academic.oup.com
While it is well appreciated that loss of the p53 tumor suppressor protein promotes cancer,
growing evidence indicates that increased p53 activity underlies the developmental defects …

A glance of p53 functions in brain development, neural stem cells, and brain cancer

Y Xiong, Y Zhang, S Xiong, AE Williams-Villalobo - Biology, 2020 - mdpi.com
p53 is one of the most intensively studied tumor suppressors. It transcriptionally regulates a
broad range of genes to modulate a series of cellular events, including DNA damage repair …

Aspm knockout ferret reveals an evolutionary mechanism governing cerebral cortical size

MB Johnson, X Sun, A Kodani, R Borges-Monroy… - Nature, 2018 - nature.com
The human cerebral cortex is distinguished by its large size and abundant gyrification, or
folding. However, the evolutionary mechanisms that drive cortical size and structure are …

High-resolution transcriptional landscape of xeno-free human induced pluripotent stem cell-derived cerebellar organoids

S Nayler, D Agarwal, F Curion, R Bowden… - Scientific Reports, 2021 - nature.com
Current protocols for producing cerebellar neurons from human pluripotent stem cells
(hPSCs) often rely on animal co-culture and mostly exist as monolayers, limiting their …

ZIKA virus elicits P53 activation and genotoxic stress in human neural progenitors similar to mutations involved in severe forms of genetic microcephaly and p53

VE Ghouzzi, FT Bianchi, I Molineris, BC Mounce… - Cell death & …, 2016 - nature.com
Epidemiological evidence from the current outbreak of Zika virus (ZIKV) and recent studies
in animal models indicate a strong causal link between ZIKV and microcephaly. ZIKV …

Pancreatic cancer survival analysis defines a signature that predicts outcome

P Raman, R Maddipati, KH Lim, A Tozeren - PloS one, 2018 - journals.plos.org
Pancreatic ductal adenocarcinoma (PDAC) is the third leading cause of cancer death in the
US. Despite multiple large-scale genetic sequencing studies, identification of predictors of …

[HTML][HTML] Poly (2-oxazoline) nanoparticle delivery enhances the therapeutic potential of vismodegib for medulloblastoma by improving CNS pharmacokinetics and …

D Hwang, T Dismuke, A Tikunov, EP Rosen… - … , Biology and Medicine, 2021 - Elsevier
We report a nanoparticle formulation of the SHH-pathway inhibitor vismodegib that improves
efficacy for medulloblastoma, while reducing toxicity. Limited blood–brain barrier (BBB) …

The neurological and non-neurological roles of the primary microcephaly-associated protein ASPM

X Wu, Z Li, ZQ Wang, X Xu - Frontiers in neuroscience, 2023 - frontiersin.org
Primary microcephaly (MCPH), is a neurological disorder characterized by small brain size
that results in numerous developmental problems, including intellectual disability, motor and …

DNA damage and repair: underlying mechanisms leading to microcephaly

JH Ribeiro, N Altinisik, N Rajan… - Frontiers in Cell and …, 2023 - frontiersin.org
DNA-damaging agents and endogenous DNA damage constantly harm genome integrity.
Under genotoxic stress conditions, the DNA damage response (DDR) machinery is crucial in …

CDC42 deletion elicits cerebral vascular malformations via increased MEKK3-dependent KLF4 expression

M Castro, B Laviña, K Ando, A Álvarez-Aznar… - Circulation …, 2019 - Am Heart Assoc
Rationale: Aberrant formation of blood vessels precedes a broad spectrum of vascular
complications; however, the cellular and molecular events governing vascular …