Goardon Nicolas, Schuh Annette, Hajar Iman, Ma Xiaoqian, Jouault Hélène, Dzierzak Elaine, Roméo Paul-Henri, Maouche-Chrétien Leïla
Institut Cochin, Département d'Hématologie, INSERM, CNRS, Paris, France.
Blood. 2002 Jul 15;100(2):491-500. doi: 10.1182/blood.v100.2.491.
The tal-1 gene encodes a basic helix-loop-helix (bHLH) transcription factor required for primitive and definitive hematopoiesis. Additionally, ectopic activation of the tal-1 gene during T lymphopoiesis occurs in numerous cases of human T-cell acute lymphoblastic leukemia. With the use of transgenic mice, we show that, in adult hematopoiesis, constitutive expression of TAL-1 protein causes disorders in the hematopoietic lineages that normally switch off tal-1 gene expression during their differentiation process. Myelopoiesis was characterized by a moderate increase of myeloid precursors and by Sca-1 antigen persistence. Although no lymphoid leukemia was observed, T lymphopoiesis and B lymphopoiesis were severely impaired. Transgenic mice showed reduced thymic cellularity together with a decrease in double-positive cells and a concurrent increase in the single-positive population. B cells exhibited a differentiation defect characterized by a reduction of the B-cell compartment most likely because of a differentiation block upstream of the intermediate pro-B progenitor. B cells escaping this defect developed normally, but transgenic splenocytes presented a defect in immunoglobulin class switch recombination. Altogether, these results enlighten the fine-tuning of TAL-1 expression during adult hematopoiesis and indicate why TAL-1 expression has to be switched off in the lymphoid lineages.
tal-1基因编码一种原始和确定性造血所需的碱性螺旋-环-螺旋(bHLH)转录因子。此外,在人类T细胞急性淋巴细胞白血病的许多病例中,T淋巴细胞生成过程中tal-1基因会发生异位激活。利用转基因小鼠,我们发现,在成体造血过程中,TAL-1蛋白的组成性表达会导致造血谱系紊乱,这些谱系在分化过程中通常会关闭tal-1基因的表达。髓系造血的特征是髓系前体细胞适度增加以及Sca-1抗原持续存在。尽管未观察到淋巴白血病,但T淋巴细胞生成和B淋巴细胞生成严重受损。转基因小鼠胸腺细胞数量减少,双阳性细胞减少,单阳性细胞群体同时增加。B细胞表现出分化缺陷,其特征是B细胞区室减少,这很可能是由于中间前B祖细胞上游的分化阻滞所致。逃脱此缺陷的B细胞发育正常,但转基因脾细胞在免疫球蛋白类别转换重组方面存在缺陷。总之,这些结果揭示了成体造血过程中TAL-1表达的精细调节,并指出了TAL-1表达在淋巴谱系中必须关闭的原因。