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PHC1

Function

Component of a Polycomb group (PcG) multiprotein PRC1-like complex, a complex class required to maintain the transcriptionally repressive state of many genes, including Hox genes, throughout development. PcG PRC1 complex acts via chromatin remodeling and modification of histones; it mediates monoubiquitination of histone H2A 'Lys-119', rendering chromatin heritably changed in its expressibility. Required for proper control of cellular levels of GMNN expression.

Involvement in disease

Microcephaly 11, primary, autosomal recessive

MCPH11

A form of microcephaly, a disease defined as a head circumference more than 3 standard deviations below the age-related mean. Brain weight is markedly reduced and the cerebral cortex is disproportionately small.

None

The disease is caused by variants affecting the gene represented in this entry.

Cellular localization

Alternative names

EDR1, PH1, PHC1, Polyhomeotic-like protein 1, hPH1, Early development regulatory protein 1

swissprot:P78364 omim:602978 entrezGene:1911