2jnj
From Proteopedia
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| - | [[Image:2jnj.gif|left|200px]] | + | [[Image:2jnj.gif|left|200px]] |
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| - | '''Solution structure of the p8 TFIIH subunit''' | + | {{Structure |
| + | |PDB= 2jnj |SIZE=350|CAPTION= <scene name='initialview01'>2jnj</scene> | ||
| + | |SITE= | ||
| + | |LIGAND= | ||
| + | |ACTIVITY= | ||
| + | |GENE= GTF2H5, TTDA ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 Homo sapiens]) | ||
| + | }} | ||
| + | |||
| + | '''Solution structure of the p8 TFIIH subunit''' | ||
| + | |||
==Overview== | ==Overview== | ||
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==About this Structure== | ==About this Structure== | ||
| - | 2JNJ is a [ | + | 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]. |
==Reference== | ==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:[http:// | + | 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:[http://www.ncbi.nlm.nih.gov/pubmed/17350038 17350038] |
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
[[Category: Single protein]] | [[Category: Single protein]] | ||
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[[Category: protein]] | [[Category: protein]] | ||
| - | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu | + | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Mar 20 17:43:13 2008'' |
Revision as of 15:43, 20 March 2008
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| Gene: | GTF2H5, TTDA (Homo sapiens) | ||||||
| Coordinates: | save as pdb, mmCIF, xml | ||||||
Solution structure of the p8 TFIIH subunit
Contents |
Overview
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.
Disease
Known disease associated with this structure: Trichothiodystrophy, complementation group A OMIM:[608780]
About this Structure
2JNJ is a Single protein structure of sequence from Homo sapiens. Full crystallographic 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
Page seeded by OCA on Thu Mar 20 17:43:13 2008
