1HYO is an EC 3.7.1.2 hydrolase cleaving C-C bonds in ketone bodies.
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Function
Disease
Mutations in 1HYO are responsible for hereditary tyrosemia Type I, a serious metabolic disease resulting in chronic inflammation of the liver and neuronal damage. It is in the same metabolic pathway as Phenylketonuria (PKU) in infants, and is treated similarly with strict dietary control and pharmacological inhibition of Phenylalanine hydroxylase, the key first enzyme in the degradation pathway.
Relevance
Structural highlights
FAH is a homodimer made up of two 46 kDa subunits. The subunits form a cavity complementary in shape and charge to fumarylacetoacetate. The binding is coordinated by Ca2+, Arg and two Tyr(INSERT IMAGE LINK). The active residues in are His-133, acting as a base to activate a water, and Arg-237, Gln-240 and Lys-253 (INSERT IMAGE LINK)acting to stabilize the tetrahedral alkoxy transition state.
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