porphobilinogen वाक्य
उदाहरण वाक्य
मोबाइल
- Hydroxymethyl bilane is a major anabolic product, from the biosynthetic reaction of porphobilinogen ( PBG ) and uroporphyrinogen I synthase ( known as porphobilinogen deaminase ).
- An example of such a conformational disease is ALAD porphyria, which results from a mutation of porphobilinogen synthase that causes a shift in its morpheein equilibrium.
- This intermediate is then attacked by the dipyrrole cofactor of porphobilinogen deaminase, which after losing a proton yields a trimer covalently bound to the enzyme ( 2 ).
- A number of substances interfere with the Ehrlich reaction on the Multistix strip : porphobilinogen, indican, p-amino salicylic acid, sulphonamide, methyldopa, procaine and chlorpromazine.
- After various failed attempts to diagnose Mark, House tests Marks urine for porphobilinogen; the test turns out positive, and Mark is diagnosed with acute intermittent porphyria ( AIP ).
- It is an autosomal recessive disorder that results from inappropriately low levels of the enzyme ALA dehydratase ( ALAD, also called porphobilinogen synthase ), which is required for normal heme synthesis.
- A dipyrrole, a cofactor of this enzyme consisting of two condensed porphobilinogen molecules, is covalently attached to domain 3 and extends into the active site, the cleft between domains 1 and 2.
- Many studies suggest that mammalian PAH shows behavior comparable to porphobilinogen synthase ( PBGS ), wherein a variety of factors such as pH and ligand binding are reported to affect enzyme activity and protein stability.
- This lone-pair effect becomes dramatic for zinc-binding proteins, such as the above-mentioned porphobilinogen synthase, as the natural substrate cannot bind anymore-in those cases the protein is inhibited.
- Several positively charged arginine residues, positioned to face the active site from domains 1 and 2, have been shown to stabilize the carboxylate functionalities on the incoming porphobilinogen as well as the growing pyrrole chain.
- The most well-known health issue involving porphobilinogen deaminase is acute intermittent porphyria, an autosomal dominant genetic disorder where insufficient hydroxymethylbilane is produced, leading to a build-up of porphobilinogen in the cytoplasm.
- The most well-known health issue involving porphobilinogen deaminase is acute intermittent porphyria, an autosomal dominant genetic disorder where insufficient hydroxymethylbilane is produced, leading to a build-up of porphobilinogen in the cytoplasm.
- Four molecules of porphobilinogen combine to form a linear tetrapyrrole, which cyclizes to uroporphyrinogen III . Oxidation and side-chain modifications lead to the synthesis of protoporphyrin IX, which acquires an iron atom to form heme.
- "' Porphobilinogen synthase "'( or "'ALA dehydratase "', or "'aminolevulinate dehydratase "') synthesizes porphobilinogen through the asymmetric condensation of two molecules of aminolevulinic acid.
- "' Porphobilinogen synthase "'( or "'ALA dehydratase "', or "'aminolevulinate dehydratase "') synthesizes porphobilinogen through the asymmetric condensation of two molecules of aminolevulinic acid.
- The one protein that is established to function as a morpheein is porphobilinogen synthase, though there are suggestions throughout the literature that other proteins may function as morpheeins ( for more information see " Table of Putative Morpheeins " below ).
- Lead ions can replace the native metal ions in several key enzymes, such as zinc cations in the ALAD enzyme, which is also known as porphobilinogen synthase, and is important in the synthesis of heme, a key component of the oxygen-carrying molecule hemoglobin.
- अधिक वाक्य: 1 2
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