Ontology highlight
ABSTRACT:
SUBMITTER: Perez-Branguli F
PROVIDER: S-EPMC6400051 | biostudies-literature | 2019 Mar
REPOSITORIES: biostudies-literature
Human molecular genetics 20190301 6
Spastic paraplegia gene 11(SPG11)-linked hereditary spastic paraplegia is a complex monogenic neurodegenerative disease that in addition to spastic paraplegia is characterized by childhood onset cognitive impairment, thin corpus callosum and enlarged ventricles. We have previously shown impaired proliferation of SPG11 neural progenitor cells (NPCs). For the delineation of potential defect in SPG11 brain development we employ 2D culture systems and 3D human brain organoids derived from SPG11 pati ...[more]