As GOF mutations in TRPV3 cause hair loss and mutilating keratoderma (see below), and since TRPV3-deficient mice display wavy hairs, curly whiskers, and a partially defective skin barrier,393,473 undisturbed TRPV3 activity appears indispensable for the proper development of the skin and skin appendages. The gene discussed is TRPV3; the disease is Palmoplantar keratoderma.