Schoenfeld N, Epstein O, Lahav M, Mamet R, Shaklai M, Atsmon A
Laboratory of Biochemical Pharmacology, Beilinson Medical Center, Petah Tiqva, Israel.
Cancer Lett. 1988 Dec 1;43(1-2):43-8. doi: 10.1016/0304-3835(88)90211-x.
The metabolism of heme is impaired in lymphocytes of patients with malignant lymphoproliferative disorders (MLPO). Two of the enzymes of the heme biosynthetic pathway, delta-aminolevulinic acid dehydrase (ALAD) (EC 4.2.1.24) and ferrochelatase (FC) (EC 4.99.1.1) are markedly reduced. The activity of porphobilinogen deaminase (PBGD) (EC 4.3.1.8) is increased. The rate-limiting enzyme of heme biosynthesis in the liver, aminolevulinate synthase (ALAS) (EC 2.3.1.37) remains unchanged although the concentration of total heme in the lymphocytes is markedly reduced. This might reflect a lack of negative feedback inhibition by heme on ALAS activity in this system.
恶性淋巴增殖性疾病（MLPO）患者淋巴细胞中的血红素代谢受损。血红素生物合成途径中的两种酶，δ-氨基乙酰丙酸脱水酶（ALAD）（EC 4.2.1.24）和亚铁螯合酶（FC）（EC 4.99.1.1）明显减少。胆色素原脱氨酶（PBGD）（EC 4.3.1.8）的活性增加。肝脏中血红素生物合成的限速酶，δ-氨基乙酰丙酸合酶（ALAS）（EC 2.3.1.37）保持不变，尽管淋巴细胞中总血红素的浓度明显降低。这可能反映了该系统中血红素对ALAS活性缺乏负反馈抑制。
