Aggregation chimeras provide evidence of in vivo intercellular correction in ovine CLN6 neuronal ceroid lipofuscinosis (Batten disease)

The neuronal ceroid lipofuscinoses (NCLs; Batten disease) are fatal, mainly childhood, inherited neurodegenerative lysosomal storage diseases. Sheep affected with a CLN6 form display progressive regionally defined glial activation and subsequent neurodegeneration, indicating that neuroinflammation m...

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Veröffentlicht in:	PloS one 2022-04, Vol.17 (4), p.e0261544-e0261544
Hauptverfasser:	Barry, Lucy Anne, Kay, Graham William, Mitchell, Nadia Lesley, Murray, Samantha Jane, Jay, Nigel P, Palmer, David Norris
Format:	Artikel
Sprache:	eng
Schlagworte:	Accumulation Agglomeration Analysis Animals Antibodies Biology and Life Sciences Brain Brain - metabolism Children Chimera - metabolism Chimeras Diagnosis Disease Embryos Genotypes Glycerol Inflammation Life sciences Lysosomal storage diseases Medicine and Health Sciences Membrane proteins Membrane Proteins - genetics Microenvironments Neurodegeneration Neurogenesis Neuronal ceroid lipofuscinosis Neuronal Ceroid-Lipofuscinoses - metabolism Neuronal-glial interactions Pathogenesis Protein binding Proteins Recovering Sheep Sheep Diseases - pathology Thickness
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