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catalytic activity:L-tyrosine + L-dopa + O(2) = L-dopa + dopaquinone + H(2)O.,cofactor:Binds 2 copper ions per subunit.,disease:Defects in TYR are the cause of oculocutaneous albinism type I temperature-sensitive (OCA-ITS) [MIM:606952]. OCA-ITS patients have white axillary and scalp hair and pigmented arm and leg hair.,disease:Defects in TYR are the cause of oculocutaneous albinism type IA (OCA-IA) [MIM:203100]. OCA-I, also known as tyrosinase negative oculocutaneous albinism, is an autosomal recessive disorder characterized by absence of pigment in hair, skin and eyes. OCA-I is divided into 2 types: type IA, characterized by complete lack of tyrosinase activity due to production of an inactive enzyme, and type IB characterized by reduced activity of tyrosinase. OCA-IA patients presents with the life-long absence of melanin pigment after birth and manifest increased sensitivity to ultrav