1ivt: Difference between revisions

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[[Image:1ivt.gif|left|200px]]<br /><applet load="1ivt" size="~~450~~" color="white" frame="true" align="right" spinBox="true"

[[Image:1ivt.gif|left|200px]]<br /><applet load="1ivt" size="350" color="white" frame="true" align="right" spinBox="true"

caption="1ivt" />

'''NMR structures of the C-terminal globular domain of human lamin A/C'''<br />

==Overview==

Lamins are nuclear intermediate filaments that, together with, lamin-associated proteins, maintain nuclear shape and provide a structural, support for chromosomes and replicating DNA. We have determined the, solution structure of the human lamin A/C C-terminal globular domain which, contains specific mutations causing four different heritable diseases., This domain encompasses residues 430-545 and adopts an Ig-like fold of, type s. We have also characterized by NMR and circular dichroism the, structure and thermostability of three mutants, R453W and R482W/Q, corresponding to "hot spots" causing Emery-Dreifuss muscular dystrophy and, Dunnigan-type lipodystrophy, respectively. Our structure determination and, mutant analyses clearly show that the consequences of the mutations, causing muscle-specific diseases or lipodystrophy are different at the, molecular level.

Lamins are nuclear intermediate filaments that, together with lamin-associated proteins, maintain nuclear shape and provide a structural support for chromosomes and replicating DNA. We have determined the solution structure of the human lamin A/C C-terminal globular domain which contains specific mutations causing four different heritable diseases. This domain encompasses residues 430-545 and adopts an Ig-like fold of type s. We have also characterized by NMR and circular dichroism the structure and thermostability of three mutants, R453W and R482W/Q, corresponding to "hot spots" causing Emery-Dreifuss muscular dystrophy and Dunnigan-type lipodystrophy, respectively. Our structure determination and mutant analyses clearly show that the consequences of the mutations causing muscle-specific diseases or lipodystrophy are different at the molecular level.

==About this Structure==

1IVT 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=1IVT OCA].

1IVT 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=1IVT OCA].

==Reference==

Line 15:

[[Category: Bonn, G.]]

[[Category: Couprie, J.]]

[[Category: Courvalin, J.C.]]

[[Category: Courvalin, J C.]]

[[Category: Gilquin, B.]]

[[Category: Hossenlopp, P.]]

[[Category: Krimm, I.]]

[[Category: Mornon, J.P.]]

[[Category: Mornon, J P.]]

[[Category: Ostlund, C.]]

[[Category: Worman, H.J.]]

[[Category: Worman, H J.]]

[[Category: Zinn-Justin, S.]]

[[Category: all sheet]]

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[[Category: ig-fold]]

''Page seeded by [http://~~ispc~~.weizmann.ac.il/oca OCA ] on ~~Tue Nov 20 17~~:42:~~34 2007~~''

''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Feb 21 13:16:14 2008''

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@@ Line 1: / Line 1: @@
-[[Image:1ivt.gif|left|200px]]<br /><applet load="1ivt" size="450" color="white" frame="true" align="right" spinBox="true"
+[[Image:1ivt.gif|left|200px]]<br /><applet load="1ivt" size="350" color="white" frame="true" align="right" spinBox="true"
 caption="1ivt" />
 '''NMR structures of the C-terminal globular domain of human lamin A/C'''<br />
 ==Overview==
-Lamins are nuclear intermediate filaments that, together with, lamin-associated proteins, maintain nuclear shape and provide a structural, support for chromosomes and replicating DNA. We have determined the, solution structure of the human lamin A/C C-terminal globular domain which, contains specific mutations causing four different heritable diseases., This domain encompasses residues 430-545 and adopts an Ig-like fold of, type s. We have also characterized by NMR and circular dichroism the, structure and thermostability of three mutants, R453W and R482W/Q, corresponding to "hot spots" causing Emery-Dreifuss muscular dystrophy and, Dunnigan-type lipodystrophy, respectively. Our structure determination and, mutant analyses clearly show that the consequences of the mutations, causing muscle-specific diseases or lipodystrophy are different at the, molecular level.
+Lamins are nuclear intermediate filaments that, together with lamin-associated proteins, maintain nuclear shape and provide a structural support for chromosomes and replicating DNA. We have determined the solution structure of the human lamin A/C C-terminal globular domain which contains specific mutations causing four different heritable diseases. This domain encompasses residues 430-545 and adopts an Ig-like fold of type s. We have also characterized by NMR and circular dichroism the structure and thermostability of three mutants, R453W and R482W/Q, corresponding to "hot spots" causing Emery-Dreifuss muscular dystrophy and Dunnigan-type lipodystrophy, respectively. Our structure determination and mutant analyses clearly show that the consequences of the mutations causing muscle-specific diseases or lipodystrophy are different at the molecular level.
 ==About this Structure==
-IVT 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=1IVT OCA].
+IVT 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=1IVT OCA].
 ==Reference==
@@ Line 15: / Line 15: @@
 [[Category: Bonn, G.]]
 [[Category: Couprie, J.]]
-[[Category: Courvalin, J.C.]]
+[[Category: Courvalin, J C.]]
 [[Category: Gilquin, B.]]
 [[Category: Hossenlopp, P.]]
 [[Category: Krimm, I.]]
-[[Category: Mornon, J.P.]]
+[[Category: Mornon, J P.]]
 [[Category: Ostlund, C.]]
-[[Category: Worman, H.J.]]
+[[Category: Worman, H J.]]
 [[Category: Zinn-Justin, S.]]
 [[Category: all sheet]]
@@ Line 27: / Line 27: @@
 [[Category: ig-fold]]
-''Page seeded by [http://ispc.weizmann.ac.il/oca OCA ] on Tue Nov 20 17:42:34 2007''
+''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Feb 21 13:16:14 2008''