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Symptoms: psychological retardation, physical disability, metabolic acidosis (because of vomiting and dehydration associated with this illness) Treatment: Limited consumption of leucine, isoleucine and valine Albinism: As talked about above Tyrosine tyrosinase melanin (a pigment of hair, skin and eyes) Melanin biosynthesis: Defected tyrosinase absent melanin albino patient Hair and skin seem white Defected vision, photophobia *excessive risk of pores and skin cancer Homocystinuria: defects in homocysteine metabolism: Cystathionine b-synthase Homocysteine Cystathione Deficiency of cystathionine b-synthase High plasma and urine ranges of homocysteine and methionine Homocysteine is a danger issue for atherosclerosis and coronary heart illness Skeletal abnormalities, osteoporosis, mental retardation, displacement of eye lens “ectopialensis” Neural tube defect (spina bifida) this end result from Deficiency of: Vascular disease (atherosclerosis) •Tetrahydrofolate Heart disease •Methioninesynthase Treatment: •Vitamin B6, B12 Oral administration of vitamins B6,B12 and folate •Folic acid Vitamin B6 is a cofactor for cystathionine b-synthase Methionine-restricted diet Alkaptonuria A uncommon disease of tyrosine degradation Due to deficiency of homogentisic acid oxidase this enzyme is required through the late stages of tyrosine degradation pathway Homogentisic acid is accumulated in tissue and cartilage?arthritis Homogentisicaciduria: elevated homogentisic acid in urine oxidized to dark urine over time And black pigmentation of cartlidge this is usually asymptomatic until maturity with arthritis as the principle complaint. 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