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1.
Sensors (Basel) ; 24(9)2024 Apr 26.
Artículo en Inglés | MEDLINE | ID: mdl-38732881

RESUMEN

Subsurface exploration of ice-covered planets and moons presents communications challenges because of the need to communicate through kilometers of ice. The objective of this task is to develop the capability to wirelessly communicate through kilometers of ice and thus complement the potentially failure-prone tethers deployed behind an ice-penetrating probe on Ocean Worlds. In this paper, the preliminary work on the development of wireless deep-ice communication is presented and discussed. The communication test and acoustic attenuation measurements in ice have been made by embedding acoustic transceivers in glacial ice at the Matanuska Glacier, Anchorage, Alaska. Field test results show that acoustic communication is viable through ice, demonstrating the transmission of data and image files in the 13-18 kHz band over 100 m. The results suggest that communication over many kilometers of ice thickness could be feasible by employing reduced transmitting frequencies around 1 kHz, though future work is needed to better constrain the likely acoustic attenuation properties through a refrozen borehole.

2.
Mol Ther Methods Clin Dev ; 32(1): 101201, 2024 Mar 14.
Artículo en Inglés | MEDLINE | ID: mdl-38374962

RESUMEN

Mucopolysaccharidosis type II (MPS II) is an X-linked recessive lysosomal disease caused by iduronate-2-sulfatase (IDS) deficiency, leading to accumulation of glycosaminoglycans (GAGs) and the emergence of progressive disease. Enzyme replacement therapy is the only currently approved treatment, but it leaves neurological disease unaddressed. Cerebrospinal fluid (CSF)-directed administration of AAV9.CB7.hIDS (RGX-121) is an alternative treatment strategy, but it is unknown if this approach will affect both neurologic and systemic manifestations. We compared the effectiveness of intrathecal (i.t.) and intravenous (i.v.) routes of administration (ROAs) at a range of vector doses in a mouse model of MPS II. While lower doses were completely ineffective, a total dose of 1 × 109 gc resulted in appreciable IDS activity levels in plasma but not tissues. Total doses of 1 × 1010 and 1 × 1011 gc by either ROA resulted in supraphysiological plasma IDS activity, substantial IDS activity levels and GAG reduction in nearly all tissues, and normalized zygomatic arch diameter. In the brain, a dose of 1 × 1011 gc i.t. achieved the highest IDS activity levels and the greatest reduction in GAG content, and it prevented neurocognitive deficiency. We conclude that a dose of 1 × 1010 gc normalized metabolic and skeletal outcomes, while neurologic improvement required a dose of 1 × 1011 gc, thereby suggesting the prospect of a similar direct benefit in humans.

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