Particularly, a subset of OCA1B mutations, including R402Q [15,16], P406L [17], and R422Q [18,19], cause the so-called temperature-sensitive oculocutaneous albinism (OCA1-TS), which leads to a temperature-sensitive form of TYR with optimal enzymatic activity at a temperature lower than 37°C; as a result, pigmentation is generally more prominent in the extremities (ears, face, and legs), where the temperature is cooler than in other parts of the body [20]. The gene discussed is TYR; the disease is oculocutaneous albinism.