592 XII. HEMOGLOBIN CATABOLISM, II 



porphyria of men; this was confirmed by the work of Fourie, Rimington, and 

 Clare {U7,022,<)23J257) . 



The so-called "acute porphyria" consists of recurrent attacks of another 

 idiopathic, actually also chronic, disease, which is inherited as a Mendelian 

 dominant {1070,2908'). The typical skin lesions of chronic porphyria are 

 absent, and the symptoms are nervous, neuromuscular, and abdominal. It 

 is not so rare, particularly in women, and is frequently misdiagnosed. The 

 urine contains varying amounts of uroporphyrin together with the dipyrrolic 

 compound porphobilinogen {cf. Chapter IV, Section 5.3.). The test for the 

 latter substance (Watson and Schwartz, 3000) is of diagnostic value. The 

 coproporphyrin content of the urine is also occasionally, but not regularly, 

 high.* No uroporphyrin is found in the bones {1914,2908). 



Data on uroporphyrin excretion are summarized in Table II, while 

 Table I gives data on the coproporphyrin excretion in porphyrias. In con- 

 genital chronic porphyria at first only uroporphyrin I was found, in acute 

 porphyria only the "uroporphyrin III" of Waldenstrom. Later, however, 

 both types were discovered to occur in both diseases, although type I appears 

 to predominate in chronic, type III in acute porphyria. 



3.3.5. Formation of Porphyrins by Bacterial Decomposition 

 of Hematin Compounds. The formation of porphyrin in the 

 intestine from hemoglobin of the diet has been studied by many 

 workers (cf. Schumm, 2^92, 2 Jf93M95M99, 2503, 2506; Papendieck, 

 2098,2099,2103; Snapper, 2583,2585; Boas, 298-300; Fischer, 7 88, 83 J^; 

 Haurowitz, 1160,1163). Protoporphyrin and deuteroporphyrin are 

 the main products. Boas {297) claimed that finding deuteroporphyrin 

 is of diagnostic value for the detection of hemorrhages in the gastro- 

 intestinal tract, but according to Hacker {108 1^) the test is of little 

 value. Agreement has not yet been reached as to the microorganisms 

 or groups of microorganisms which transform hemoglobin to proto- 

 porphyrin (1396,U2Jf,lU9). 



By prolonged putrefaction of meat Schumm and Mertens {2510) 

 and Fischer and Lindner (851) obtained deuteroporphyrin in addition 

 to protoporphyrin. Bacteria are thus able to remove the two vinyl 

 side chains of protoporphyrin. Coproporphyrin has also been found 

 in traces, but not always (24-94.). 



The autolysis of meat has been investigated by Hoagland {1296), Schumm 

 {2494) and Fischer and co-workers {840,848,876). In acid autolysis much 

 protoporphyrin was found, in autolysis in alkaline buffer much less. Copro- 

 porphyrin has only been found after several weeks of autolysis, particularly 

 in alkaline buffers. In such experiments of long duration bacterial infection 

 is hard to exclude and the coproporphyrin may have been formed by bacterial 

 synthesis. 



* The porphyrins are often excreted as zinc complexes. 



