| porphyric polyneuropathy |
Polyneuropathy resulting from acute porphyria, characterized by pains and paresthesias in the extremities and by flaccid paralysis.
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| porphin |
a heterocyclic structure composed of four pyrrole rings connected by methylidyne (—CH=) bridges; it is the parent skeleton of the porphyrins.
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| porphobilinogen |
the immediate precursor of the porphyrins, a pyrrole ring with acetyl, propionyl, and aminomethyl side chains; four molecules of porphobilinogen are condensed to form one molecule of uroporphyrinogen III, which is then converted successively to coproporphyrinogen III, protoporphyrin IX, and heme. Porphobilinogen is produced in excess and excreted in the urine in acute intermittent porphyria and several other porphyrias. Abbreviated PBG.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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| porphobilinogen deaminase |
hydroxymethylbilane synthase.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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| porphobilinogen synthase |
[EC 4.2.1.24] an enzyme of the lyase class that catalyzes the condensation of two molecules of δ-aminolevulinate to form porphobilinogen in the synthesis of porphyrins. The enzyme is inhibited by minute quantities of lead poisoning. Genetic deficiency of the enzyme causes a porphyria similar to acute intermittent porphyria but with high urinary levels of δ-aminolevulinic acid, coproporphyrinogen III, and protoporphyrin but not porphobilinogen. Called also aminolevulinate dehydratase.
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