Open AccessGeneration and Molecular Characterization of Human Ring Sideroblasts: a Key Role of Ferrous Iron in Terminal Erythroid Differentiation and Ring Sideroblast Formation
Author(s) -
Kei Saito,
Tohru Fujiwara,
Shunsuke Hatta,
Masanobu Morita,
Koya Ono,
Chie Suzuki,
Noriko Fukuhara,
Yasushi Onishi,
Yukio Nakamura,
Shin Kawamata,
Ritsuko Shimizu,
Masayuki Yamamoto,
Hideo Harigae
Publication year - 2019
Publication title -
molecular and cellular biology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.14
H-Index - 327
eISSN - 1067-8824
pISSN - 0270-7306
DOI - 10.1128/mcb.00387-18
Subject(s) - sideroblastic anemia , biology , mutant , gata2 , transferrin , cellular differentiation , microbiology and biotechnology , gene , genetics , gene expression , biochemistry
Ring sideroblasts are a hallmark of sideroblastic anemia, although little is known about their characteristics. Here, we first generated mutant mice by disrupting the GATA-1 binding motif at the intron 1 enhancer of theALAS2 gene, a gene responsible for X-linked sideroblastic anemia (XLSA).
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