Tyrosinemia
From Biocrawler, the free encyclopedia.
Tyrosinemia is an error of metabolism, usually inborn, in which the body can not effectively break down the amino acid tyrosine, found in most animal and plant proteins.
Tyrosinemia is caused by an absence of the enzyme fumarylacetoacetate hydrolase (FAH) which is essential in the metabolism of tyrosine, which takes place primarily in the liver.
The absence of FAH leads to an accumulation of toxic metabolic products in various body tissues, which in turn results in progressive damage to the liver and kidneys.
Treatment varies depending on the specific type. A low protein diet may be required in the management of tyrosinemia.
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