53 research outputs found

    C9ORF72 patient-derived endothelial cells drive blood-brain barrier disruption and contribute to neurotoxicity

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    The blood-brain barrier (BBB) serves as a highly intricate and dynamic interface connecting the brain and the bloodstream, playing a vital role in maintaining brain homeostasis. BBB dysfunction has been associated with multiple neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS); however, the role of the BBB in neurodegeneration is understudied. We developed an ALS patient-derived model of the BBB by using cells derived from 5 patient donors carrying C9ORF72 mutations. Brain microvascular endothelial-like cells (BMEC-like cells) derived from C9ORF72-ALS patients showed altered gene expression, compromised barrier integrity, and increased P-glycoprotein transporter activity. In addition, mitochondrial metabolic tests demonstrated that C9ORF72-ALS BMECs display a significant decrease in basal glycolysis accompanied by increased basal and ATP-linked respiration. Moreover, our study reveals that C9-ALS derived astrocytes can further affect BMECs function and affect the expression of the glucose transporter Glut-1. Finally, C9ORF72 patient-derived BMECs form leaky barriers through a cell-autonomous mechanism and have neurotoxic properties towards motor neurons

    Open data from the third observing run of LIGO, Virgo, KAGRA, and GEO

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    The global network of gravitational-wave observatories now includes five detectors, namely LIGO Hanford, LIGO Livingston, Virgo, KAGRA, and GEO 600. These detectors collected data during their third observing run, O3, composed of three phases: O3a starting in 2019 April and lasting six months, O3b starting in 2019 November and lasting five months, and O3GK starting in 2020 April and lasting two weeks. In this paper we describe these data and various other science products that can be freely accessed through the Gravitational Wave Open Science Center at https://gwosc.org. The main data set, consisting of the gravitational-wave strain time series that contains the astrophysical signals, is released together with supporting data useful for their analysis and documentation, tutorials, as well as analysis software packages

    Complex fragment production and multifragmentation in 139^{139}La-induced reactions at 35, 40, 45 and 55 MeV/u

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    Complex fragment emission (Z\u3e3) has been studied in the reactions of 35, 40, 45 and 55 MeV/u139La+X. Charge, angular, and energy distributions were measured inclusively and in coincidence with other complex fragments, and were used to extract source rapidities, velocity distributions, and cross sectins. Multifragment events increase with both bombarding energy and entrance-channel mass asymmetry. The excitation functions for multifragment events rise strongly with excitation energy. These excitation functions are independent of the target-projectile combination and bombarding energy suggesting, the formation of an intermediate nuclear system, whose decay properties depend mainly on its excitation energy and angular momentum
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