Sandbox Reserved 768: Difference between revisions
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PAH enzyme is present in the liver cells of humans and other mammals. It is also present in non-mammalian eukaryote organisms and some bacteria such as ''E.coli''. <ref name= "pdb"/>. Recently, it has been identified in some protozoans and slime molds, and even in nonflowering plants such as spinach from which it has been extracted and studied. <ref> Nair, P, and L Vining. "Phenylalanine Hydroxylase from Spinach Leaves." Phytochemistry 4.3 (1965): 401-411. Web. </ref>. | PAH enzyme is present in the liver cells of humans and other mammals. It is also present in non-mammalian eukaryote organisms and some bacteria such as ''E.coli''. <ref name= "pdb"/>. Recently, it has been identified in some protozoans and slime molds, and even in nonflowering plants such as spinach from which it has been extracted and studied. <ref> Nair, P, and L Vining. "Phenylalanine Hydroxylase from Spinach Leaves." Phytochemistry 4.3 (1965): 401-411. Web. </ref>. | ||
Mammalian PAH is a homo-tetrameric enzyme of 50 kDa subunits composed of two asymmetric dimeric units. The two dimers are connected to each other via a coiled-coil motif. | Mammalian PAH is a homo-tetrameric enzyme of 50 kDa subunits composed of two asymmetric dimeric units. The two dimers are connected to each other via a coiled-coil motif. | ||
[[Image:PAH tetramer complex.jpg]] | |||
Each monomeric subunit is composed of three sites: the N-terminal, the catalytic site, and the C-terminal. <ref name= "flydal"/>. | Each monomeric subunit is composed of three sites: the N-terminal, the catalytic site, and the C-terminal. <ref name= "flydal"/>. | ||
PAH enzyme has been studied extensively because of its correlation with the genetic defective condition phenylketonuria (PKU). Errors in the function or stability of PAH lead to its malfunction which causes a buildup of phenylalanine resulting in numerous health detriments. | PAH enzyme has been studied extensively because of its correlation with the genetic defective condition phenylketonuria (PKU). Errors in the function or stability of PAH lead to its malfunction which causes a buildup of phenylalanine resulting in numerous health detriments. | ||