Full size table A few opalescent pale foci could be observed on the epicardial surface of the heart, and were present as early as 10 months of age in an untreated affected dog (Figure 7a). Some foci were observed in each of the older affected treated dogs that were necropsied, and eith...
including anaphylaxis and when to seek immediate medical care. If a severe allergic reaction (e.g., anaphylaxis) occurs during infusion, the infusion should be stopped immediately, and your child should receive medical attention. Contact your doctor or get medical help right away if ...
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Full size image These data indicate that the 45 kDa and 65–80 kDa CLN3 protein forms represent the newly synthesized and the mature form respectively, with the latter undergoing heavy glycosylation and trafficking through the Golgi and lysosomal compartments. CLN3 interacts with protein complexe...
Conversely, in dogs receiving the high dose of TPP1-producing MSCs, the treated eye exhibited a full preservation of the ERG through 7 months of age. There was no significant difference between the b-wave amplitude of treated eyes and normal, age-matched eyes until 8 months of age ...
Download: Download full-size image Figure 1. Infection of CLN7-deficient cells with SARS-CoV-2 (A) Infection with SARS-CoV-2 for approximately 48 h revealed that CLN7 knockout cells (line 99-4) had a 10-fold reduction in viral load compared to HEK293T wild-type cells. (B) This exp...
Scale bar: 250 μm in a, 50 μm in b-i Full size image Fig. 7 Ultrastructural morphology of neuronal storage material. Semi-thin section stained with toluidin blue of a neuron in the spinal cord anterior horn of patient 2 shows strongly stained storage material (a). Ultrastructural...
Full size image Severe failure to develop in CLN3Q352XhiPSC-derived cerebral organoids Little is known about early brain development in the context of JNCL. To evaluate whether there is an early developmental component to CLN3 disease, we used cerebral organoids as a state of the art human ...
You have full access to thisopen accessarticle Acta Neuropathologica Communications Gemma Gomez-Giro, Jonathan Arias-Fuenzalida, Javier Jarazo, Dagmar Zeuschner, Muhammad Ali, Nina Possemis, Silvia Bolognin, Rashi Halder, Christian Jäger,
Full size image Table 1 Editing the sheep PPT1 locus. Full size table Confirmation of CLN1 phenotype A one step fluorometric PPT1 enzyme activity assay4 was employed in wild type, heterozygote and asymptomatic homozygote PPT1 sheep plasma at approximately 1 year of age (Fig. 2). There was ...