The inversin compartment (InvsC) comprises the products of four genes, INVS/NPHP2, NPHP3, NEK8/NPHP9 and ANKS6/NPHP16, all of which have been implicated in infantile-onset NPH in addition to juvenile and/or adult NPH depending on the severity of the mutations (Gagnadoux et al., 1989; Olbrich et al., 2003; Otto et al., 2003, 2008; Bergmann et al., 2008; Tory et al., 2009; Hoff et al., 2013; Rajagopalan et al., 2015; Grampa et al., 2016). Here, INVS is linked to normal pressure hydrocephalus.