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1H6F

Human TBX3, a transcription factor responsible for ulnar-mammary syndrome, bound to a palindromic DNA site

1H6F の概要
エントリーDOI10.2210/pdb1h6f/pdb
分子名称T-BOX TRANSCRIPTION FACTOR TBX3, 5'-D(*TP*AP*AP*TP*TP*TP*CP*AP*CP*AP*CP*CP*TP* AP*GP*GP*TP*GP*TP*GP*AP*AP*AP*T)-3', MAGNESIUM ION, ... (4 entities in total)
機能のキーワードtranscription factor, tbx3, t-box transcription factor, ulnar-mammary syndrome, holt-oram-syndrome, ig-fold, dna-binding, repressor, nuclear protein, developmental protein
由来する生物種HOMO SAPIENS (HUMAN)
詳細
細胞内の位置Nucleus : O15119
タンパク質・核酸の鎖数4
化学式量合計60156.62
構造登録者
Coll, M.,Muller, C.W. (登録日: 2001-06-13, 公開日: 2002-04-19, 最終更新日: 2023-12-13)
主引用文献Coll, M.,Seidman, J.G.,Muller, C.W.
Structure of the DNA-Bound T-Box Domain of Human Tbx3, a Transcription Factor Responsible for Ulnar- Mammary Syndrome
Structure, 10:343-, 2002
Cited by
PubMed Abstract: T-box genes encode transcription factors involved in morphogenesis and organogenesis of vertebrates and invertebrates. Mutations in human T-box genes TBX3, TBX5, and TBX1 cause severe genetic disorders known as Ulnar-Mammary syndrome (UMS), Holt-Oram syndrome (HOS), and DiGeorge syndrome, respectively. The crystal structure of the T-box domain of the first human T-box transcription factor, TBX3, in complex with DNA at 1.7 A resolution explains structural consequences of T-box domain point mutations observed in UMS and HOS patients. Comparison with the structure of the T-box domain from Xenopus laevis (Xbra) bound to DNA shows differences in several secondary structure elements and in the quaternary structure of the two complexes. TBX3 independently recognizes the two binding sites present in the palindromic DNA duplex, whereas in Xbra, binding to the palindrome is stabilized through interactions between the two monomers. The different quaternary structures suggest different DNA binding modes for T-box transcription factors.
PubMed: 12005433
DOI: 10.1016/S0969-2126(02)00722-0
主引用文献が同じPDBエントリー
実験手法
X-RAY DIFFRACTION (1.7 Å)
構造検証レポート
Validation report summary of 1h6f
検証レポート(詳細版)ダウンロードをダウンロード

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件を2026-04-22に公開中

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