However, longer primary cilia with abnormal Hh signaling were evoked by the aberrant expression of XPNPEP3 in either patient urine sediment cells or kidney tubular epithelial cells of Xpnpep3-KO mice, emphasizing the major role of XPNPEP3 homeostasis in mitochondrial function and ciliopathy. The gene discussed is XPNPEP3; the disease is ciliopathy.