2jnj: Difference between revisions

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[[Image:2jnj.gif|left|200px]]<br />

[[Image:2jnj.gif|left|200px]]<br /><applet load="2jnj" size="350" color="white" frame="true" align="right" spinBox="true"

<applet load="2jnj" size="~~450~~" color="white" frame="true" align="right" spinBox="true"

caption="2jnj" />

'''Solution structure of the p8 TFIIH subunit'''<br />

==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.

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==

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==About this Structure==

2JNJ is a [http://en.wikipedia.org/wiki/Single_protein Single protein] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://~~ispc~~.weizmann.ac.il/oca-bin/ocashort?id=2JNJ OCA].

2JNJ is a [http://en.wikipedia.org/wiki/Single_protein 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==

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://ispc.weizmann.ac.il//pmbin/getpm?pmid=17350038 17350038]

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://ispc.weizmann.ac.il//pmbin/getpm?pmid=17350038 17350038]

[[Category: Homo sapiens]]

[[Category: Single protein]]

[[Category: Atkinson, R.A.]]

[[Category: Atkinson, R A.]]

[[Category: Kieffer, B.]]

[[Category: Moras, D.]]

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[[Category: protein]]

''Page seeded by [http://~~ispc~~.weizmann.ac.il/oca OCA ] on ~~Mon Nov 12 22~~:56:~~41 2007~~''

''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Feb 21 18:04:24 2008''

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@@ Line 1: / Line 1: @@
-[[Image:2jnj.gif|left|200px]]<br />
+[[Image:2jnj.gif|left|200px]]<br /><applet load="2jnj" size="350" color="white" frame="true" align="right" spinBox="true"
-<applet load="2jnj" size="450" color="white" frame="true" align="right" spinBox="true"
 caption="2jnj" />
 '''Solution structure of the p8 TFIIH subunit'''<br />
 ==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.
+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==
@@ Line 11: / Line 10: @@
 ==About this Structure==
-JNJ is a [http://en.wikipedia.org/wiki/Single_protein Single protein] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://ispc.weizmann.ac.il/oca-bin/ocashort?id=2JNJ OCA].
+JNJ is a [http://en.wikipedia.org/wiki/Single_protein 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==
-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://ispc.weizmann.ac.il//pmbin/getpm?pmid=17350038 17350038]
+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://ispc.weizmann.ac.il//pmbin/getpm?pmid=17350038 17350038]
 [[Category: Homo sapiens]]
 [[Category: Single protein]]
-[[Category: Atkinson, R.A.]]
+[[Category: Atkinson, R A.]]
 [[Category: Kieffer, B.]]
 [[Category: Moras, D.]]
@@ Line 24: / Line 23: @@
 [[Category: protein]]
-''Page seeded by [http://ispc.weizmann.ac.il/oca OCA ] on Mon Nov 12 22:56:41 2007''
+''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Feb 21 18:04:24 2008''