Morc2a, short for Microrchidia CW-type zinc finger 2, is related to multiple biological processes. It is involved in DNA repair, adipogenesis, and epigenetic silencing via the human silencing hub (HUSH) complex [2]. Additionally, it plays a role in the repression of retroelements in mouse embryonic stem cells [4].

In Morc2a p.S87L mutant mice, which mimic human Charcot-Marie-Tooth disease type 2Z (CMT2Z), there are peripheral and central neuropathies. The p.S87L mutation causes a protein synthesis defect, leading to loss of Morc2a function, high cellular apoptosis induced by high hydroxyl radical levels, DNA damage accumulation in neuronal cells, and subsequent p53/cytochrome c/caspase 9/caspase 3-mediated apoptosis [1,2]. Inactivation of Morc2a in the nervous system of mice leads to increased brain size, altered brain architecture, and behavioral changes, as it suppresses the repetitive-like protocadherin gene cluster in an H3K9me3-dependent manner [3].

In conclusion, Morc2a is crucial for normal neural function, playing a role in processes that prevent DNA damage-induced apoptosis in neurons. The Morc2a p.S87L mutant mouse models have been instrumental in revealing the mechanisms underlying CMT2Z and related neuropathies, providing potential targets for therapeutic intervention in these incurable genetic disorders [1,2].