Characterization of a novel hiPSC-derived neuronal model of CLN12 Batten disease
Batten disease encompasses a heterogenous group of 13 genetically distinct neurodegenerative lysosomal storage disorders. The group shares many clinical phenotypes including progressive intellectual and motility disability, myoclonic epilepsy, blindness, and cerebral atrophy before eventual patient death. My aim was to establish a novel model of CLN12 Batten disease in human neurons for the assessment of pathological hallmarks. I generated a novel line of iPSC derived CLN12 deficient neuronal cultures to provide accurate modeling of human cell biology. Knockdown of CLN12 coincided with increased α-synuclein deposition, inhibited lysosomal trafficking, changes in total neurite length, increased lysosomal cathepsin activity, decreased mitochondrial membrane potential and health and loss of dendritic synapse density. Collectively, these findings establish a human iPSC model of CLN12 deficiency as a viable tool for further research.
Zoom
Zoom link for Oluwatobi Eboda's Biochemistry seminar
Zoom password: bioc
| Date | Tuesday, 21 March 2023 |
|---|---|
| Time | 12:00pm - 1:00pm |
| Audience | Undergraduate students,Postgraduate students,Staff,Invite only |
| Event Category | Health Sciences |
| Event Type |
Seminar Academic Online and in-person |
| Campus | Dunedin |
| Department | Biochemistry |
| Location | Biochemistry Seminar Room G.13 (BIG13), Dunedin and online via Zoom |
| Cost | Free |
| Contact Name | Department of Biochemistry |
| Contact Email | biochemistry@otago.ac.nz |
