Porphyrin synthesis and movement across human and rabbit red cell membrane with delta aminolaevulinic acid as substrate

Characterization of the Porphobilinogen Deaminase Deficiency in Acute Intermittent Porphyria

“…'. 'This would suggest that the induction of hepatic 8-aminolevulinate synthase during an acute attack resulted in the increased production of 8-aminolevulinic acid and PBG (32) that gained access to the erythrocyte where the PBG became bound to the PBGdeaminase (33). Thus, the patterns of the PBGdeaminase intermediates in AIP heterozygotes were consistent with the expression of the normal allele and were dependent, in part, on the substrate concentration in erythrocytes.…”

Light-induced depigmentation in planarians models the pathophysiology of acute porphyrias

“…The uniform fluorescence in the anterior (top) corresponds to recently regenerated tissue (animals were photographed 3.5 weeks after cephalic amputation). Fluorescence does not appear to be restricted to pigment cells, particularly within regenerated tissue; this may reflect porphyrin movement across cell membranes ( Viljoen et al, 1975 ). ( C ) Whole-animal H 2 SO 4 homogenates were photographed in plastic cuvettes over a black (400 nm) LED.…”