Taurine deficiency in experimental animals produces degeneration of light-sensitive cells. Low levels of taurine are found in retinitis pigmentosa. It is often difficult to distinguish compensatory changes in human biochemistry from true metabolic or deficiency disease. One reason that the findings are not entirely clear is that taurine is often elevated in the blood of epileptics who need it. Taurine levels have been found to decrease significantly in many depressed patients. Cysteine and B6 are the nutrients most directly involved in taurine synthesis. The amino acids alanine and glutamic acid, as well as pantothenic acid, inhibit taurine metabolism while vitamins A and B6, zinc, and manganese help build taurine. Taurine in the brain is usually associated with zinc or manganese. It also lowers glutamic acid in the brain, and preliminary clinical trials suggest taurine may be useful in some forms of epilepsy. Its inhibitory effect is one source of taurine's anticonvulsant and antianxiety properties. Taurine, after GABA, is the second most important inhibitory neurotransmitter in the brain. In two with mitral valve prolapse, congestive cardiomyopathy eventually developed while the amounts of urinary taurine doubled (OMIM: 145350 ). Other family members had late or holosystolic mitral valve prolapse and elevated urinary taurine values (about 2.5 times normal). Hypertaurinuric cardiomyopathy describes congestive cardiomyopathy and markedly elevated urinary taurine levels (about 5 times normal). Parkinsonism developed later, and respiratory failure occurred terminally (OMIM: 168605 ). Sleep disturbances, exhaustion, and marked weight loss were features. The earliest and most prominent symptom was mental depression that was not responsive to antidepressant drugs or electroconvulsive therapy. Symptoms began late in the fifth decade in 6 affected persons and death occurred after 4 to 6 years. Perry syndrome is an unusual neuropsychiatric disorder inherited in an autosomal dominant fashion through three generations of a family. Several inborn errors of taurine metabolism have been described. Deficiency of taurine occurs in premature infants, neonates fed formula milk, and various disease states. It can be synthesized by the body from cysteine when vitamin B6 is present. Taurine is highly concentrated in animal and fish protein, which are good sources of dietary taurine. Taurine has many diverse biological functions including serving as a neurotransmitter in the brain, a stabilizer of cell membranes, and a facilitator in the transport of ions such as sodium, potassium, calcium, and magnesium. Taurine is abundant in the brain, heart, breast, gallbladder, and kidney and has important roles in health and disease in these organs. Adults can synthesize their own taurine, yet are probably dependent, in part, on dietary taurine. Yet taurine is an essential amino acid in pre-term and newborn infants of humans and many other species. It is a lesser-known amino acid because it is not incorporated into the structural building blocks of protein. Taurine is a sulfur amino acid like methionine, cystine, cysteine, and homocysteine. Enzymes (11) Show 11 proteins Record Information
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