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PDBsum entry 2ko0
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Transcription/DNA
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PDB id
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2ko0
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Contents |
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* Residue conservation analysis
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Nucleic Acids Res
38:3466-3476
(2010)
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PubMed id:
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Structural determinants of specific DNA-recognition by the THAP zinc finger.
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S.Campagne,
O.Saurel,
V.Gervais,
A.Milon.
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ABSTRACT
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Human THAP1 is the prototype of a large family of cellular factors sharing an
original THAP zinc-finger motif responsible for DNA binding. Human THAP1
regulates endothelial cell proliferation and G1/S cell-cycle progression,
through modulation of pRb/E2F cell-cycle target genes including rrm1. Recently,
mutations in THAP1 have been found to cause DYT6 primary torsion dystonia, a
human neurological disease. We report here the first 3D structure of the complex
formed by the DNA-binding domain of THAP1 and its specific DNA target (THABS)
found within the rrm1 target gene. The THAP zinc finger uses its double-stranded
beta-sheet to fill the DNA major groove and provides a unique combination of
contacts from the beta-sheet, the N-terminal tail and surrounding loops toward
the five invariant base pairs of the THABS sequence. Our studies reveal
unprecedented insights into the specific DNA recognition mechanisms within this
large family of proteins controlling cell proliferation, cell cycle and
pluripotency.
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Literature references that cite this PDB file's key reference
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PubMed id
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Reference
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D.C.Bragg,
I.A.Armata,
F.C.Nery,
X.O.Breakefield,
and
N.Sharma
(2011).
Molecular pathways in dystonia.
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Neurobiol Dis,
42,
136-147.
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L.J.Ozelius,
and
S.B.Bressman
(2011).
Genetic and clinical features of primary torsion dystonia.
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Neurobiol Dis,
42,
127-135.
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F.J.Kaiser,
A.Osmanoric,
A.Rakovic,
A.Erogullari,
N.Uflacker,
D.Braunholz,
T.Lohnau,
S.Orolicki,
M.Albrecht,
G.Gillessen-Kaesbach,
C.Klein,
and
K.Lohmann
(2010).
The dystonia gene DYT1 is repressed by the transcription factor THAP1 (DYT6).
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Ann Neurol,
68,
554-559.
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The most recent references are shown first.
Citation data come partly from CiteXplore and partly
from an automated harvesting procedure. Note that this is likely to be
only a partial list as not all journals are covered by
either method. However, we are continually building up the citation data
so more and more references will be included with time.
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