3jt0: Difference between revisions
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===Crystal Structure of the C-terminal fragment (426-558) Lamin-B1 from Homo sapiens, Northeast Structural Genomics Consortium Target HR5546A=== | |||
=== | ==Disease== | ||
[[http://www.uniprot.org/uniprot/LMNB1_HUMAN LMNB1_HUMAN]] Defects in LMNB1 are the cause of leukodystrophy demyelinating autosomal dominant adult-onset (ADLD) [MIM:[http://omim.org/entry/169500 169500]]. ADLD is a slowly progressive and fatal demyelinating leukodystrophy, presenting in the fourth or fifth decade of life. Clinically characterized by early autonomic abnormalities, pyramidal and cerebellar dysfunction, and symmetric demyelination of the CNS. It differs from multiple sclerosis and other demyelinating disorders in that neuropathology shows preservation of oligodendroglia in the presence of subtotal demyelination and lack of astrogliosis.<ref>PMID:16951681</ref> | |||
==Function== | |||
[[http://www.uniprot.org/uniprot/LMNB1_HUMAN LMNB1_HUMAN]] Lamins are components of the nuclear lamina, a fibrous layer on the nucleoplasmic side of the inner nuclear membrane, which is thought to provide a framework for the nuclear envelope and may also interact with chromatin. | |||
==About this Structure== | ==About this Structure== | ||
[[3jt0]] is a 2 chain structure with 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=3JT0 OCA]. | |||
==Reference== | |||
<references group="xtra"/><references/> | |||
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
[[Category: Abashidze, M.]] | [[Category: Abashidze, M.]] | ||
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[[Category: Tong,L.]] | [[Category: Tong,L.]] | ||
[[Category: Xiao,R.]] | [[Category: Xiao,R.]] | ||
[[Category: Hr5546a]] | [[Category: Hr5546a]] | ||
[[Category: Intermediate filament]] | [[Category: Intermediate filament]] | ||
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[[Category: Nucleus]] | [[Category: Nucleus]] | ||
[[Category: Phosphoprotein]] | [[Category: Phosphoprotein]] | ||
[[Category: Prenylation]] | [[Category: Prenylation]] | ||
[[Category: Protein structure initiative]] | [[Category: Protein structure initiative]] | ||
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[[Category: Structural genomic]] | [[Category: Structural genomic]] | ||
[[Category: Structural protein]] | [[Category: Structural protein]] | ||
Revision as of 01:36, 25 March 2013
Crystal Structure of the C-terminal fragment (426-558) Lamin-B1 from Homo sapiens, Northeast Structural Genomics Consortium Target HR5546A
Disease
[LMNB1_HUMAN] Defects in LMNB1 are the cause of leukodystrophy demyelinating autosomal dominant adult-onset (ADLD) [MIM:169500]. ADLD is a slowly progressive and fatal demyelinating leukodystrophy, presenting in the fourth or fifth decade of life. Clinically characterized by early autonomic abnormalities, pyramidal and cerebellar dysfunction, and symmetric demyelination of the CNS. It differs from multiple sclerosis and other demyelinating disorders in that neuropathology shows preservation of oligodendroglia in the presence of subtotal demyelination and lack of astrogliosis.[1]
Function
[LMNB1_HUMAN] Lamins are components of the nuclear lamina, a fibrous layer on the nucleoplasmic side of the inner nuclear membrane, which is thought to provide a framework for the nuclear envelope and may also interact with chromatin.
About this Structure
3jt0 is a 2 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.
Reference
Proteopedia Page Contributors and Editors (what is this?)
Categories:
- Homo sapiens
- Abashidze, M.
- Acton, T B.
- Belote,R L.
- Ciccosanti,C.
- Everett, J K.
- Hunt,J F.
- Kuzin,A.
- Montelione, G T.
- NESG, Northeast Structural Genomics Consortium.
- Nair, R.
- Rost, B.
- Sahdev,S.
- Seetharaman, J.
- Tong,L.
- Xiao,R.
- Hr5546a
- Intermediate filament
- Lamin-b1
- Leukodystrophy
- Lipoprotein
- Lmnb1 human
- Membrane
- Nesg
- Northeast structural genomics consortium
- Nucleus
- Phosphoprotein
- Prenylation
- Protein structure initiative
- Psi-2
- Structural genomic
- Structural protein
