Ichikawa H, Shimizu K, Hayashi Y, Ohki M
Radiobiology Division, National Cancer Center Research Institute, Tokyo, Japan.
Cancer Res. 1994 Jun 1;54(11):2865-8.
The t(16;21)(p11;q22) translocation is a recurrent chromosomal abnormality found in several types of myeloid leukemia. We have previously demonstrated that the breakpoints of this translocation are clustered in a specific intron of the ERG gene on chromosome 21, which has recently been reported to be involved in Ewing's sarcoma. We show here that the TLS/FUS gene on chromosome 16 is fused with the ERG gene to produce the TLS/FUS-ERG chimeric transcript by this translocation. The TLS/FUS gene has been identified as a translocated gene in myxoid liposarcoma by the t(12;16)(q13;p11) translocation and encodes an RNA-binding protein that is highly homologous to the product of the EWS gene involved in Ewing's sarcoma. Thus, the TLS/FUS-ERG gene fusion in t(16;21) leukemia is predicted to produce a protein that is very similar to the EWS-ERG chimeric protein responsible for Ewing's sarcoma.
t(16;21)(p11;q22)易位是在几种髓系白血病中发现的一种常见染色体异常。我们之前已经证明，这种易位的断点聚集在21号染色体上ERG基因的一个特定内含子中，最近有报道称该内含子与尤因肉瘤有关。我们在此表明，16号染色体上的TLS/FUS基因通过这种易位与ERG基因融合，产生TLS/FUS-ERG嵌合转录本。TLS/FUS基因已通过t(12;16)(q13;p11)易位被鉴定为黏液样脂肪肉瘤中的一个易位基因，它编码一种RNA结合蛋白，该蛋白与尤因肉瘤中涉及的EWS基因的产物高度同源。因此，预计t(16;21)白血病中的TLS/FUS-ERG基因融合会产生一种与导致尤因肉瘤的EWS-ERG嵌合蛋白非常相似的蛋白质。
