Xiao-E Jia, Ke Ma, Tao Xu, Lei Gao, Shuang Wu, Cong Fu, Wenjuan Zhang, Zhizhang Wang, Kaiyu Liu, Mei Dong, Changbin Jing, Chunguang Ren, Zhiwei Dong, Yi Chen, Yi Jin, Qiuhua Huang, Xing Chang, Min Deng, Li Li, Lingfei Luo, Jun Zhu, Yongjun Dang, Hung-Chun Chang, Leonard I Zon, Yi Zhou, Saijuan Chen, Weijun Pan
Dysregulation of ribosome biogenesis causes human diseases, such as Diamond-Blackfan anemia, del (5q-) syndrome and bone marrow failure. However, the mechanisms of blood disorders in these diseases remain elusive. Through genetic mapping, molecular cloning and mechanism characterization of the zebrafish mutant cas002, we reveal a novel connection between ribosomal dysfunction and excessive autophagy in the regulation of hematopoietic stem/progenitor cells (HSPCs). cas002 carries a recessive lethal mutation in kri1l gene that encodes an essential component of rRNA small subunit processome...
August 2015: Cell Research