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DSB repair through HR process is an error free pathway, since, it requires an extensive region of sequence homology between the damaged and template strands, whereas NHEJ is an error prone, alternate pathway for the repair of DSBs, essentially joins broken chromosomal ends independent of sequence homology

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In Pelizaeus-Merzbacher disease, a myelination defect, a subgroup of mutations affecting the extracellular loop of the PLP/DM20 protein impair formation of intramolecular disulfide bridges and cause abnormal protein cross-links, ER retention and oligodendrocyte death (Dhaunchak et al., 2005) and von Willebrand disease (Wang et al., 2012)