By U. A. Meyer (auth.), Prof. Dr. Manfred Doss (eds.)
On the celebration of the 450th anniversary of Philipp collage, the porphyrin examine workforce of. the dept of scientific Biochemistry within the school of medication prepared a world symposium. This was once held among June 28 and July 1, 1977, below the joint chairman send of Professor Samuel Schwartz and the editor. The organizers and chairmen of the symposium desire to thank the Sozialminister of Hessen, Armin Clauss, the President of the German Society for scientific Chemis try out, Professor A. Delbrlick, and the Dean of the school of drugs of Philipp collage, Professor F. Neurath, for his or her help and patronage. we're additionally thankful to Professor A. Treibs of the Institute of natural Chemistry, Technical college of Munich. Professor Treibs, a former student, co-worker and now successor to the Nobel laureate Hans Fischer, kindly authorised the honorary presidency of this overseas symposium. the outlet rite was once inaugurated by means of the chairmen, with welcoming speeches through the President of Philipps college, Mr. Rudolf Zingel and consumers of the collage. The editor defined the ten years of improvement of medical biochemistry at Philipp collage and traced intimately the continuous improvement of its leader examine attempt in porphyria. It was once mentioned that following the 1st overseas symposium at the law of porphyrin and heme biosynthesis in 1973 at Marburg (Doss, M. (ed. ): legislation of Porphyrin and Heme Biosyn thesis.
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Extra resources for Diagnosis and Therapy of Porphyrias and Lead Intoxication: International Symposium Clinical Biochemistry
This finding is of relevance to the present study, because in the present experiments in which there were no cytosolic proteins, one would expect the intramitochondrial accumulation of deuteroheme. As shown in Table 2, ~ 80% of the deuteroheme synthesized by intact mitochondria during 20 min incubation remained bound to the mitochondria. However, by increasing the temperature or disrupting the mitochondrial membranes, there was a marked release of deuteroheme to the suspending medium. Thus, endogenously synthesized deuteroheme apparently did not freely penetrate to the suspending medium.
4 trace a). Essentially similar results were obtained when the mitochondria were pre incubated with iron followed by CCCP prior to deuteroporphyrin (Fig. 4 traces c and d). However, when the reactants were added in this sequence, the ferrochelatase activity could not be restored by X537A. These results therefore not only point to iron as an important metabolite to be transported across the inner membrane, but also reveal an energy requirement for the influx of deuteroporphyrin as well. , compared to the accmuulation of deuteroporphyrin by intact mitochondria, there was a reduction in the amount of deuteroporphyrin accumulated by de-energized and K+-depleted mitochondria.
These constellations of results are limited in time. During the course (progression and remission) of the disease - with exception of uro-S activity - these parameters may vary considerably in the same patient. The following are some representative cases: 33 Table 4. , female, 24, with acute syndrome with skin symptoms (May, 1976) : PV Urine nmol/24 h Uro 1903 Normal range Feces nmol/g Normal range 4-29 30 1-7 Hepta 130 0-4 5 0-3 Hexa 62 0-3 3 0-1 7 0-4 Penta 137 Copro 901 (83%III) Tri Di Total porphyrin 0-6 21-119 63 5-37 38 0-2 44 0-16 7 0-1 648 3178 21-151 836 llmol/24 h ALA 119 2-49 PBG 228 1-8 uro-s Ery [llmol/liter/h (% of controls)) 59(108) 1.
Diagnosis and Therapy of Porphyrias and Lead Intoxication: International Symposium Clinical Biochemistry by U. A. Meyer (auth.), Prof. Dr. Manfred Doss (eds.)