One of the hedgehog family of conserved signalling molecules that coordinate various patterning processes during early embryonic development. Desert hedgehog (Dhh, 396 aa) is restricted, in the developing mouse, to Sertoli cells of developing testes and Schwann cells of peripheral nerves. The precursor molecule is autocatalytically cleaved and the C-terminal product covalently attaches a cholesterol moiety to the soluble N-terminal product that contains the signalling activity, restricting it to the cell surface. Defects in Dhh have been associated with partial gonadal dysgenesis (PGD) accompanied by minifascicular polyneuropathy.
Subjects: Medicine and Health.