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Table 3 BSC studies models of proteopathic inclusion pathologies beyond Aβ and tau

From: Organotypic brain slice cultures to model neurodegenerative proteinopathies

Human Pathology

Slice Culture Pathology

Reference(s)

α-synuclein inclusions

rAAV expression of WT or A53T human α-synuclein in BSCs induces pser129 α-synuclein inclusions. rAAV expression of A53T human α-synuclein in nigrostriatal circuit BSCs also develop α-synuclein inclusions.

[29, 82]

SOD-1 inclusions

SOD-1 accumulation in spinal cord slice cultures from transgenic G93A SOD-1 mice from 3 weeks in culture. SOD-1 inclusions also inducible in G85R SOD-1 transgenic spinal cord cultures with human SOD-1 seeds from spinal tissues progressively over a 20 day incubation.

[85, 86]

TDP-43 inclusions

BSCs from rats expressing human mutant TDP-43 develop TDP-43 inclusions and associated micro- and astrogliosis from 10 DIV.

[87]

Huntingtin inclusions

Biolistic transfection of human wild-type and mutant huntingtin (HTT) in striatal and cortical rat BSCs results in the development of HTT inclusions by 7 DIV.

[89]