プロリダーゼ欠損症患者, 母親及び正常人赤血球中のプロリダーゼの精製とその性質
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概要
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Two forms of prolidase could be separated from erythrocytes of normal subjects and mother of patients with prolidase deficiency using TSK DEAE-5PW Chromatography. Only one from of prolidase was separated from erythrocytes of patients with prolidase defi-ciency. Each peak ( I and II) of prolidase differed in their response to Mn^<2+>, substrate specificity and heat stability. Prolidase activity in erythrocytes from patients with prolidase deficiency showed complete lack of peak I of normal prolidase, whereas peak 11 had normal activity against all the substrate tested. The various properties between patients' prolidase and peak II of normal prolidase were very similar. Prolidase I (EC 3.4.13.9) have been purified to homogeneity from the erythrocytes of normal human and patient's mother. Prolidase I from normal human and patient's mother have as molecular weight of about 112,000 and are composed of two subunits identical in molecular weight of 56,000. The Km values from gly-pro of normal and patient mother's prolidase I were 2.90 and 2.88 mM, but the Vmax values for gly-pro of mother's enzyme was reduced about 30% compared to that of normal enzymes (mother : 6.02units/mg protein, normal : 22.2lunits/mg protein). Isoionic points of those enzymes by chromatofocusing were pH 4.6-4.7. Prolidase 11 from erythrocytes of normal human, and patient's mother, and proiidase from patient's erythrocytes have also been purified highly. Prolidase II from normal human, patient's mother and patient's prolidase have a molecular weight of about 185,000,and are composed of two subunits identical in molecular weight of 95,000. The Km and Vmax values for various substrates of these enzymes are almost same.
- 1988-12-10