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Interrelationship of Pyrrole and Globin Metabolism in β‐Thalassaemia *
Author(s) -
KreimerBirnbaum Martha,
Bannerman R. M.
Publication year - 1968
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.1968.tb01508.x
Subject(s) - labelling , globin , pigment , red cell , glycine , metabolism , urine , biochemistry , heme , biology , urinary system , chemistry , medicine , hemoglobin , endocrinology , amino acid , enzyme , organic chemistry
Detailed metabolic studies in a case of β‐thalassaemia intermedia following 14 C‐2‐glycine administration demonstrate some of the interrelationships of pyrrole pigments and haemoglobins, and some secondary effects of a β‐thalassaemia mutation. Marked increase in the early‐labelled fraction of faecal bile pigment was confirmed; labelling rose to a maximum at 5 days, in advance of the haem peak, and was approximately three times greater than maximum haem labelling. Urinary coproporphyrins were increased in quantity, thus allowing complete serial determinations of radioactivity, which was at its maximum within 24 hours. The urine also contained a brown pigment, probably a dipyrrole, which had a labelling pattern resembling that of the urinary coproporphyrins, with maximum activity in the first sample. It is suggested that the ‘dipyrrole’may arise by early destruction of redundant haem associated with α‐chains. At all times, haemoglobin F was more highly labelled than haemoglobin A in both the haem and the globin moieties. Mean cell survival estimated graphically from the various curves was approximately 32 days. Haem/globin ratios were similar to those reported previously in normal subjects and in thalassaemia.