These findings did not exclude the role of LIM2, PAX6, PITX3 and HSF4 gene in both ocular and central nervous system but it underlined the fact that these transcription factors (PAX6, PITX3), heat-shock transcription factor (HSF4) and lens intrinsic membrane protein (LIM2) could not be responsible for the association between ARCC, MR and ARCC, MR and microcephaly in the four studied Tunisian families in spite of their expression in the human brain (PAX6, PITX3, HSF4) or their interaction with proteins expressed in human brain (LIM2). The gene discussed is HSF4; the disease is microcephaly.