Neurolipid Biology Group, Instituto de Biologia Molecular e Celular (IBMC), Instituto de Investigação e Inovação em Saúde (i3S), University of Porto, Porto, Portugal.
Nerve Regeneration Group, Instituto de Biologia Molecular e Celular (IBMC), Instituto de Investigação e Inovação em Saúde (i3S), University of Porto, Porto, Portugal.
PLoS Biol. 2022 Jul 6;20(7):e3001706. doi: 10.1371/journal.pbio.3001706. eCollection 2022 Jul.
In this issue of PLOS Biology, Kreher and colleagues show in a mouse model that in vivo, neurons and not only myelinating glia are primary effectors of disease progression in Krabbe disease. The neuron-specific model generated allows the unprecedented capacity to investigate the neuronal autonomous component of this disorder.
在本期《公共科学图书馆·生物学》杂志中,Kreher 及其同事在小鼠模型中表明,在体内,神经元而非仅仅是髓鞘形成胶质细胞是脑苷脂沉积病疾病进展的主要效应器。该神经元特异性模型的产生使得对这种疾病的神经元自主成分进行前所未有的研究成为可能。