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Title: Deficiency of smarcal1 causes cell cycle arrest and developmental abnormalities in zebrafish
Author: Huang, Cheng ; Gu, Shanye ; Yu, Pengchun ; Yu, Fudong ; Feng, Chun ; Gao, Ning ; null(杜久林)
Source: DEVELOPMENTAL BIOLOGY
Issued Date: 2010
Volume: 339, Issue:1, Pages:89-100
Keyword: Cell cycle ; Chromatin remodeling ; Development ; Schimke Immuno-Osseous Dysplasia smarcal1 ; Apoptosis ; IMMUNO-OSSEOUS DYSPLASIA ; HUMAN-DISEASE ; EMBRYONIC HEMATOPOIESIS ; TRANSCRIPTION FACTORS ; E2F FAMILY ; T-CELL ; EXPRESSION ; MODEL ; GENES ; DIFFERENTIATION
Subject: Developmental Biology
Corresponding Author: Huang, C (reprint author), Shanghai Univ Tradit Chinese Med, Sch Pharm, 1200 Cailun Rd, Shanghai 201203, Peoples R China,chuang@sibs.ac.cn ; forestdu@ion.ac.cn
English Abstract: Mutations in SMARCAL1 cause Schimke Immuno-Osseous Dysplasia (SIOD), an autosomal recessive multisystem developmental disease characterized by growth retardation, T-cell deficiency, bone marrow failure, anemia and renal failure. SMARCAL1 encodes an ATP-driven annealing helicase. However, the biological function of SMARCAL1 and the molecular basis of SIOD remain largely unclear. In this work, we cloned the zebrafish homologue of the human SMARCAL1 gene and found that smarcal1 regulated cell cycle progression. Morpholino knockdown of smarcal1 in zebrafish recapitulated developmental abnormalities in SIOD patients, including growth retardation, craniofacial abnormality, and haematopoietic and vascular defects. Lack of smarcal1 caused G0/G1 cell cycle arrest and induced cell apoptosis. Furthermore, using Electrophoretic Mobility Shift Assay and reporter assay, we found that SMARCAL1 was transcriptionally inhibited by E2F6, an important cell cycle regulator. Over-expression of E2F6 in zebrafish embryos reduced the expression of smarcal1 mRNA and induced developmental defects similar to those in smarcal1 morphants. These results suggest that SIOD may be caused by defects in cell cycle regulation. Our study provides a model of SIOD and reveals its cellular and molecular bases. (C) 2009 Elsevier Inc. All rights reserved.
Indexed Type: sci
Language: 英语
Content Type: 期刊论文
URI: http://ir.sibs.ac.cn/handle/331001/1602
Appears in Collections:神经所(总)_期刊论文
感觉整合与行为研究组_期刊论文

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Huang, Cheng; Gu, Shanye; Yu, Pengchun; Yu, Fudong; Feng, Chun; Gao, Ning; Du, Jiulin.Deficiency of smarcal1 causes cell cycle arrest and developmental abnormalities in zebrafish,DEVELOPMENTAL BIOLOGY,2010,339(1):89-100
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