Liver biopsy confirms hepatic disease in EPP by the presence of protoporphyrin deposits in the hepatocytes that can be observed as a brown pigment within the biliary canaliculi and the portal macrophages.
32.
Zinc protoporphyrin, formed in developing red blood cells instead of hemoglobin when iron is unavailable or lead is present, has a bright fluorescence and can be used to detect these problems.
33.
In less than five percent of cases, accumulation of protoporphyrin in the liver results in cholestasis ( blockage of bile flow from the liver to the small intestine ) and terminal liver failure.
34.
Protoporphyrinogen oxidase is responsible for the seventh step in biosynthesis of protoporphyrin IX . This porphyrin is the precursor to hemoglobin, the oxygen carrier in animals, and chlorophyll, the dye in plants.
35.
Ferrochelatase catalyzes the insertion of ferrous iron into protoporphyrin IX in the heme biosynthesis pathway to form heme B . The enzyme is localized to the matrix-facing side of the inner mitochondrial membrane.
36.
The hydrophobic lips, consisting of the highly conserved residues 300 311, face the inner mitochondrial membrane and facilitate the passage of the poorly soluble protoporphyrin IX substrate and the heme product via the membrane.
37.
"' Magnesium-chelatase "'is a three-component enzyme that catalyses the insertion of Mg 2 + into protoporphyrin IX . This is the first unique step in the synthesis of bacteriochlorophyll.
38.
It was already known at this time that non-heme Protoporphyrin IX levels were elevated in these conditions, but prior investigators had used acidic extraction methods in their assays that converted ZPP to unbound Protoporphyrin IX.
39.
It was already known at this time that non-heme Protoporphyrin IX levels were elevated in these conditions, but prior investigators had used acidic extraction methods in their assays that converted ZPP to unbound Protoporphyrin IX.
40.
Examinations of eggs attributed to " Heyuannia " by Martin Sanders " et al " revealed the eggs had high levels of biliverdin and protoporphyrin, indicating they were a blue-green in color.