7DW5

Crystal structure of DUX4 HD1-HD2 domain complexed with ERG sites

Summary for 7DW5

• Entry DOI: 10.2210/pdb7dw5/pdb
• Descriptor: Double homeobox protein 4-like protein 2, DNA (5'-D(P*CP*GP*AP*CP*TP*TP*GP*AP*TP*GP*AP*GP*AP*TP*TP*AP*GP*AP*CP*TP*G)-3'), BROMIDE ION, ... (5 entities in total)
• Functional Keywords: acute lymphoblastic leukemia, dux4/igh, ergalt, clamp-like transactivation, dux4-responsive-element, alternative splicing, dna binding protein, dna binding protein-dna complex, dna binding protein/dna
• Biological source: Homo sapiens (Human); More
• Total number of polymer chains: 6
• Total formula weight: 60816.48

Authors

Zhang, H.,Cheng, N.,Li, Z.,Zhang, W.,Dong, X.,Huang, J.,Meng, G. (deposition date: 2021-01-15, release date: 2021-11-03, Last modification date: 2023-11-29)

Primary citation

Zhang, H.,Cheng, N.,Li, Z.,Bai, L.,Fang, C.,Li, Y.,Zhang, W.,Dong, X.,Jiang, M.,Liang, Y.,Zhang, S.,Mi, J.,Zhu, J.,Zhang, Y.,Chen, S.J.,Zhao, Y.,Weng, X.Q.,Hu, W.,Chen, Z.,Huang, J.,Meng, G.
DNA crosslinking and recombination-activating genes 1/2 (RAG1/2) are required for oncogenic splicing in acute lymphoblastic leukemia.
Cancer Commun (Lond), 41:1116-1136, 2021

PubMed Abstract: Abnormal alternative splicing is frequently associated with carcinogenesis. In B-cell acute lymphoblastic leukemia (B-ALL), double homeobox 4 fused with immunoglobulin heavy chain (DUX4/IGH) can lead to the aberrant production of E-26 transformation-specific family related gene abnormal transcript (ERG ) and other splicing variants. However, the molecular mechanism underpinning this process remains elusive. Here, we aimed to know how DUX4/IGH triggers abnormal splicing in leukemia.

PubMed: 34699692
DOI: 10.1002/cac2.12234

Experimental method

X-RAY DIFFRACTION (2.83 Å)