Neurofibromatosis type 2 (NF2) is caused by inactivation of the NF2 gene, which encodes merlin. NF2 patients develop peripheral neuropathies. The authors show that NF2 inactivation decreases axonal integrity in mice and NF2 patient tissue. Their data suggest that merlin activates RhoA and promotes neurofilament heavy chain phosphorylation to maintain axonal integrity.
- Alexander Schulz
- Stephan L Baader
- Helen Morrison