菅野 仁
   Department   School of Medicine(Tokyo Women's Medical University Hospital), School of Medicine
   Position   Professor (Fixed Term)
Article types Original article
Language English
Peer review Peer reviewed
Title Gene expression and biological significance of hexokinase in erythroid cells.
Journal Formal name:Acta haematologica
Abbreviation:Acta Haematol
ISSN code:0001-5792(Print)0001-5792(Linking)
Volume, Issue, Page 108(4),pp.204-9
Author and coauthor Murakami Koko, Kanno Hitoshi, Tancabelic Jakica, Fujii Hisaichi
Authorship 2nd author
Publication date 2002/09
Summary Red blood cells (RBCs) express two hexokinase (HK) isoforms, HK-I and HK-R. Both isozymes are generated from the HK-I gene by use of an alternate promoter. Gene structure and exon-intron organization of the HK-I gene have been elucidated from a sequence of three contiguous genomic clones localized at human chromosome10. The sequence spans about 131 kb, and consists of 25 exons, which include 6 testis- and 1 erythroid-specific exons. HK-R has been shown as an erythroid-specific isozyme whose expression is turned on in the early erythroid-progenitors and is significantly induced during their differentiation. HK-R unfolds major HK activity in immature RBCs and is rapidly degraded during the maturation process. HK-I has a porin-binding domain in its N-terminus. Recent studies have shown that HK isozymes with a porin-binding domain play a role in mitochondrial integrity, suggesting that HK-I-deficient erythroid cells might be eliminated by apoptosis. It is most likely that RBCs are most labile as a result of HK-I/R deficiency since the HK-I gene but not the other isozyme genes are expressed in fetal and adult RBCs.
DOI 65656
Document No. 12432216