Results 161 to 170 of about 20,553 (236)

The novel Cln1(R151X) mouse model of infantile neuronal ceroid lipofuscinosis (INCL) for testing nonsense suppression therapy. [PDF]

Hum Mol Genet, 2015
Miller JN, Kovács AD, Pearce DA.
europepmc   +1 more source

Reimagining State Aid: Guiding Law and Policy Reform in a Changing World

European Law Journal, EarlyView.
ABSTRACT EU state aid law has shifted fundamentally from an originally purely repressive tool to a proactive steering mechanism for critical EU goals today. This paper argues that the current legal framework in Article 107 TFEU is gravely misaligned with this new function.
Thomas Jaeger
wiley   +1 more source

Nonsense-mediated mRNA decay: friend or foe in cancer biology? [PDF]

Cell Commun Signal
Wang Y, He C, Chen C, Ma X, Gao X, Li G, Li T.   +6 more
europepmc   +1 more source

Directional information flow in human frataxin defines allosteric pathways connecting the hydrophobic core to the iron‐binding ridge

The FEBS Journal, EarlyView.
Human frataxin deficiency causes Friedreich's ataxia, yet how iron‐binding events are communicated across the protein is unclear. Using transfer entropy analysis of molecular dynamics simulations, we identify buried hydrophobic core leucines (LEU136, LEU140) as the source of directional signaling toward the iron‐binding acidic ridge.
Kevser Kübra Kırboğa, Ecir Uğur Küçüksille   +1 more
wiley   +1 more source

Identification of small molecules that enhance aminoglycoside-mediated suppression of <i>CFTR</i> and <i>NF1</i> nonsense mutations. [PDF]

Mol Ther Oncol
Sammons J, Chen J, Thrasher K, Fu L, Du M, Wen H, Bostwick JR, Vinson P, Moukha-Chafiq O, Augelli-Szafran C, Keeling KM, Wallis D, Kesterson RA, Rowe SM, Bedwell DM.   +14 more
europepmc   +1 more source

Newly identified human aminoacyl‐tRNA synthetase complex interacting multifunctional protein 2 (AIMP2) loss‐of‐function mutations cause neurodevelopmental defects linked to cell death in a zebrafish model

The FEBS Journal, EarlyView.
Human AIMP2 mutations lead to severe neurodevelopmental defects and brain atrophy. Using patient‐derived fibroblasts from two individuals, we show decreased AIMP2 protein levels and overall protein synthesis. In a zebrafish loss‐of‐function model, the lack of AIMP2 leads to an increase in cell death and results in smaller brains.
Patrick Mullen, Shenela Lakhani, Joshua Appelbaum, DéJenaé See, Pryce Patterson, Collin M. MacLeod, Alexandra George, Reza Maroofian, Margaret E. Ross, Christopher Francklyn, Alicia M. Ebert   +10 more
wiley   +1 more source

Choosing Startup Investors: Does Gender Matter?

Financial Management, EarlyView.
ABSTRACT We investigate whether startups respond differently to male and female investors during outbound origination, a setting where female investors often report being marginalized by male founders. Using a randomized field experiment, we examine gender differences in startups' responses to investor outreach.
Ofir Gefen, David Reeb, Johan Sulaeman
wiley   +1 more source

Functional allelic diversity of PTGS components revealed by a high-throughput RUBY-based screening. [PDF]

Plant J
Wei X, Hou Y, Si Y, Guo W, Qiu Y, Li H, Sun Y, Tang Z, Mu L, Yan W, Guo H.   +10 more
europepmc   +1 more source

Not all crickets are equal: selective shrike predation highlights the conservation value of diverse arthropod communities to grassland birds

Ibis, EarlyView.
Understanding dietary preferences and prey selection is pivotal for interpreting the ecology and conservation needs of threatened species. We investigated the diet composition and prey selection of the Red‐backed Shrike Lanius collurio in a montane grassland of the central Italian Apennines, with special regard to sex‐related differences and the ...
Leonardo Ancillotto, Sauro Giannerini, Matteo Bianchi, Giulia Santalmasi, Marco Zaccaroni, Tommaso Lipparelli, Matteo Annessi, Jacopo Martino, Emiliano Mori   +8 more
wiley   +1 more source

Tetrahedral DNA nanostructure-delivered suppressor tRNA ameliorates nephropathy in <i>COL4A5</i> nonsense mutation-mediated Alport syndrome mice. [PDF]

NAR Mol Med
Lv J, Li Y, Hong D, Shen Y, Mi Z, Liang Q, Zheng Z, Kong B, Gao W, Dou Z, Tian S, Xia Q.   +11 more
europepmc   +1 more source