The heme biosynthetic pathway in lymphocytes of patients with malignant lymphoproliferative disorders.
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.
Schoenfeld
N
N
Laboratory of Biochemical Pharmacology, Beilinson Medical Center, Petah Tiqva, Israel.
Epstein
O
O
Lahav
M
M
Mamet
R
R
Shaklai
M
M
Atsmon
A
A
D000624:5-Aminolevulinate Synthetase / Q000097:blood; D005294:Ferrochelatase / Q000097:blood; D006418:Heme / Q000096:biosynthesis*; D006801:Humans; D011163:Hydroxymethylbilane Synthase / Q000097:blood; D007700:Kinetics; D008214:Lymphocytes / Q000378:metabolism*; D008232:Lymphoproliferative Disorders / Q000097:blood*; D000623:Porphobilinogen Synthase / Q000097:blood
D006418:Heme; D000624:5-Aminolevulinate Synthetase; D011163:Hydroxymethylbilane Synthase; D000623:Porphobilinogen Synthase; D005294:Ferrochelatase