Abstract
The effect of some sulphydryl compounds on two new variants of red cell pyruvate kinase (ATP: pyruvate phosphotransferase, PK) is reported. In vitro a striking correction has been obtained of both the qualitative and, in one case, the quantitative defect of red cell PK. In vivo, a correction of the qualitative and quantitative abnormalities has been produced in both patients, with clinical improvement of one of them. These findings, together with the unexpected results with respect to the functional properties of PK found in the affected members of the two families studied, suggest that the PK abnormality is not the cause of the haemolytic anaemia, but an epiphenomenon of a primary unknown defect that apparently involves the red cell thiol groups.
| Original language | English |
|---|---|
| Pages (from-to) | 373-385 |
| Number of pages | 13 |
| Journal | British Journal of Haematology |
| Volume | 32 |
| Issue number | 3 |
| Publication status | Published - 1976 |
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ASJC Scopus subject areas
- Hematology
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Effects of sulphydryl compounds on abnormal red cell pyruvate kinase. / Zanella, A.; Rebulla, P.; Giovanetti, A. M.; Curzio, M.; Pescarmona, G. P.; Sirchia, G.
In: British Journal of Haematology, Vol. 32, No. 3, 1976, p. 373-385.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Effects of sulphydryl compounds on abnormal red cell pyruvate kinase
AU - Zanella, A.
AU - Rebulla, P.
AU - Giovanetti, A. M.
AU - Curzio, M.
AU - Pescarmona, G. P.
AU - Sirchia, G.
PY - 1976
Y1 - 1976
N2 - The effect of some sulphydryl compounds on two new variants of red cell pyruvate kinase (ATP: pyruvate phosphotransferase, PK) is reported. In vitro a striking correction has been obtained of both the qualitative and, in one case, the quantitative defect of red cell PK. In vivo, a correction of the qualitative and quantitative abnormalities has been produced in both patients, with clinical improvement of one of them. These findings, together with the unexpected results with respect to the functional properties of PK found in the affected members of the two families studied, suggest that the PK abnormality is not the cause of the haemolytic anaemia, but an epiphenomenon of a primary unknown defect that apparently involves the red cell thiol groups.
AB - The effect of some sulphydryl compounds on two new variants of red cell pyruvate kinase (ATP: pyruvate phosphotransferase, PK) is reported. In vitro a striking correction has been obtained of both the qualitative and, in one case, the quantitative defect of red cell PK. In vivo, a correction of the qualitative and quantitative abnormalities has been produced in both patients, with clinical improvement of one of them. These findings, together with the unexpected results with respect to the functional properties of PK found in the affected members of the two families studied, suggest that the PK abnormality is not the cause of the haemolytic anaemia, but an epiphenomenon of a primary unknown defect that apparently involves the red cell thiol groups.
UR - http://www.scopus.com/inward/record.url?scp=0017230089&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0017230089&partnerID=8YFLogxK
M3 - Article
C2 - 1252372
AN - SCOPUS:0017230089
VL - 32
SP - 373
EP - 385
JO - British Journal of Haematology
JF - British Journal of Haematology
SN - 0007-1048
IS - 3
ER -