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1h9d

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1h9d, resolution 2.60Å ()
Gene: AML1 (Homo sapiens), CBFB (Homo sapiens)
Domains: CBF_beta, Runt
Resources: FirstGlance, OCA, PDBsum, RCSB
Coordinates: save as pdb, mmCIF, xml



Contents

AML1/CBF-BETA/DNA COMPLEX

Publication Abstract from PubMed

We have determined the structure, at 2.6 A resolution, of the AML1 (Runx1) Runt domain--CBF beta--DNA ternary complex, the most common target for mutations in human leukemia. The structure reveals that the Runt domain DNA binding mechanism is unique within the p53 family of transcription factors. The extended C-terminal 'tail' and 'wing' elements adopt a specific DNA-bound conformation that clamps the phosphate backbone between the major and minor grooves of the distorted B-form DNA recognition site. Furthermore, the extended 'tail' mediates most of the NF-kappa B/Rel-like base-specific contacts in the major groove. The structure clearly explains the molecular basis for the loss of DNA binding function of the Runt domain--CBF beta complex as a consequence of the human disease-associated mutations in leukemogenesis and cleidocranial dysplasia.

The leukemia-associated AML1 (Runx1)--CBF beta complex functions as a DNA-induced molecular clamp., Bravo J, Li Z, Speck NA, Warren AJ, Nat Struct Biol. 2001 Apr;8(4):371-8. PMID:11276260

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.

Disease

Known disease associated with this structure: Leukemia, acute myeloid OMIM:[151385], Platelet disorder, familial, with associated myeloid malignancy OMIM:[151385], Rheumatoid arthritis, susceptibility to OMIM:[151385], Myeloid leukemia, acute, M4Eo subtype OMIM:[121360]

About this Structure

1H9D is a 8 chains structure of sequences from Homo sapiens. Full crystallographic information is available from OCA.

Reference

  • Bravo J, Li Z, Speck NA, Warren AJ. The leukemia-associated AML1 (Runx1)--CBF beta complex functions as a DNA-induced molecular clamp. Nat Struct Biol. 2001 Apr;8(4):371-8. PMID:11276260 doi:10.1038/86264

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