MCPH1 (microcephalin) binds condensin II complex through direct interaction with NCAPG2 and possibly NCAPD3 condensin II subunits (Wood et al. 2008, Yamashita et al. 2011). MCPH1 binding sequesters condensin II by preventing loading of condensin II on chromatin. Simultaneous binding of MCPH1 to the SET oncogene may contribute to condensin II sequestering (Leung et al. 2011). Mutations in MCPH1 are a cause of microchephaly inhereted in an autosomally recessive manner. MCPH1 deficient cells show premature chromosome condensation (PCC) phenotype, with metaphase-like chromosomes apparent in prophase, before nuclear envelope breakdown (Wood et al. 2008).
Leung, JW, Leitch, A, Wood, JL, Shaw-Smith, C, Metcalfe, K, Bicknell, LS, Jackson, AP, Chen, J
Yamashita, D, Shintomi, K, Ono, T, Gavvovidis, I, Schindler, D, Neitzel, H, Trimborn, M, Hirano, T
Wood, JL, Liang, Y, Li, K, Chen, J
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