ALS-FTD-linked CCNFS621G drives increased hippocampal astrocyte ramification and mitochondrial dysfunction and impairs motor neuron excitability

Liam Robinson, Dzung Do-Ha, Flora Cheng, Claire H. Stevens, Rossana Rosa Porto, Madilyn Coles, Jamesha Subachandran, Predrag Kalajdzic, Joanna Lui, Rachelle Balez, Sonia Sanz Muñoz, Mauricio Castro Cabral-da-Silva, Tracey Berg, Marco Morsch, Leszek Lisowski, Rachel H. Tan, Gaétan Burgio, Tim Karl, A · 2026

Read ALS-FTD-linked CCNFS621G drives increased hippocampal astrocyte ramification and mitochondrial dysfunction and impairs motor neuron excitability by Liam Robinson, Dzung Do-Ha, Flora Cheng, Claire H. Stevens, Rossana Rosa Porto, Madilyn Coles, Jamesha Subachandran, Predrag Kalajdzic, Joanna Lui, Rachelle Balez, Sonia Sanz Muñoz, Mauricio Castro Cabral-da-Silva, Tracey Berg, Marco Morsch, Leszek Lisowski, Rachel H. Tan, Gaétan Burgio, Tim Karl, A (2026) in the Tower of Babel, a free online library of public-domain works preserved in clean, searchable text. No sign-up. The full text is served as a plain-text file at a predictable URL, where text is present: https://antarctic-labs.com/tower-of-babel/pmc-13289496.txt.

Description

Two diseases, ALS and frontotemporal dementia, share roots, and faults in a gene called CCNF can cause either one. Liam Robinson and co-authors build the first mouse carrying the disease-linked change at its natural level, rather than flooded in from outside. They track the animals for 18 months.

The mice showed no movement decline and no loss of nerve cells. Brain scans of tissue, though, showed star-shaped support cells with more branches than normal in the memory region. Protein surveys pointed to early strain in energy, structure, and signaling systems. Human stem-cell models echoed the pattern: support cells had weak energy membranes, and motor nerve cells grown alone fired too easily. Similar support cell changes appeared in patient tissue after death. The results suggest the gene fault primes support cells early, before clear damage.

Catalog record

CREATORLiam Robinson, Dzung Do-Ha, Flora Cheng, Claire H. Stevens, Rossana Rosa Porto, Madilyn Coles, Jamesha Subachandran, Predrag Kalajdzic, Joanna Lui, Rachelle Balez, Sonia Sanz Muñoz, Mauricio Castro Cabral-da-Silva, Tracey Berg, Marco Morsch, Leszek Lisowski, Rachel H. Tan, Gaétan Burgio, Tim Karl, A
YEAR2026
COLLECTIONPAPERS
FORMATTXT
FILE SIZE94 KB
VERSION1.0
LICENSECC BY 4.0
RIGHTSCC BY
SOURCEEurope PMC

Source: https://europepmc.org/articles/PMC13289496

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