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2jnj

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[[Image:2jnj.gif|left|200px]]
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{{STRUCTURE_2jnj| PDB=2jnj | SCENE= }}
{{STRUCTURE_2jnj| PDB=2jnj | SCENE= }}
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'''Solution structure of the p8 TFIIH subunit'''
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===Solution structure of the p8 TFIIH subunit===
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==Overview==
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Trichothiodystrophy (TTD) is a rare hereditary multi-system disorder associated with defects in nucleotide excision repair (NER) and transcription as consequences of mutations in XPB, XPD and p8/TTD-A subunits of transcription factor IIH (TFIIH). Here, we report the solution structure of the p8/TTD-A protein, a small alpha/beta protein built around an antiparallel beta-sheet that forms a homodimer with an extended interface. In order to characterize the dimer interface, we have introduced a mutation at position 44, which destabilizes the dimeric form of the protein. We have shown that this mutation has no effect on the intrinsic ability of p8/TTD-A to stimulate NER in vitro, but affects the capacity of p8/TTD-A to restore TFIIH concentration in TTD-A fibroblasts. Point mutations found in TTD-A patients are discussed on the basis of the present structure.
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(as it appears on PubMed at http://www.pubmed.gov), where 17350038 is the PubMed ID number.
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{{ABSTRACT_PUBMED_17350038}}
==About this Structure==
==About this Structure==
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2JNJ is a [[Single protein]] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2JNJ OCA].
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2JNJ is a [[Single protein]] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2JNJ OCA].
==Reference==
==Reference==
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[[Category: Vitorino, M.]]
[[Category: Vitorino, M.]]
[[Category: Protein]]
[[Category: Protein]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Sun May 4 09:04:35 2008''
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Mon Jul 28 16:30:08 2008''

Revision as of 13:30, 28 July 2008

Image:2jnj.png

Template:STRUCTURE 2jnj

Solution structure of the p8 TFIIH subunit

Template:ABSTRACT PUBMED 17350038

About this Structure

2JNJ is a Single protein structure of sequence from Homo sapiens. Full experimental information is available from OCA.

Reference

Solution structure and self-association properties of the p8 TFIIH subunit responsible for trichothiodystrophy., Vitorino M, Coin F, Zlobinskaya O, Atkinson RA, Moras D, Egly JM, Poterszman A, Kieffer B, J Mol Biol. 2007 Apr 27;368(2):473-80. Epub 2007 Feb 20. PMID:17350038

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