1jvk
From Proteopedia
(New page: 200px<br /><applet load="1jvk" size="450" color="white" frame="true" align="right" spinBox="true" caption="1jvk, resolution 1.94Å" /> '''THREE-DIMENSIONAL ST...) |
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| - | [[Image:1jvk.gif|left|200px]]<br /><applet load="1jvk" size=" | + | [[Image:1jvk.gif|left|200px]]<br /><applet load="1jvk" size="350" color="white" frame="true" align="right" spinBox="true" |
caption="1jvk, resolution 1.94Å" /> | caption="1jvk, resolution 1.94Å" /> | ||
'''THREE-DIMENSIONAL STRUCTURE OF AN IMMUNOGLOBULIN LIGHT CHAIN DIMER ACTING AS A LETHAL AMYLOID PRECURSOR'''<br /> | '''THREE-DIMENSIONAL STRUCTURE OF AN IMMUNOGLOBULIN LIGHT CHAIN DIMER ACTING AS A LETHAL AMYLOID PRECURSOR'''<br /> | ||
==Overview== | ==Overview== | ||
| - | The X-ray structure of an immunoglobulin light-chain dimer isolated from | + | The X-ray structure of an immunoglobulin light-chain dimer isolated from the urine as a "Bence-Jones protein" from a patient with multiple myeloma and amyloidosis (Sea) was determined at 1.94 A resolution and refined to R and R(free) factors of 0.22 and 0.25, respectively. This "amyloidogenic" protein crystallized in the orthorhombic P2(1)2(1)2(1) space group with unit-cell parameters a = 48.28, b = 83.32, c = 112.59 A as determined at 100 K. In the vital organs (heart and kidneys), the equivalent of the urinary protein produced fibrillar amyloid deposits which were fatal to the patient. Compared with the amyloidogenic Mcg light-chain dimer, the Sea protein was highly soluble in aqueous solutions and only crystallized at concentrations approaching 100 mg ml(-1). Both the Sea and Mcg proteins packed into crystals in highly ordered arrangements typical of strongly diffracting crystals of immunoglobulin fragments. Overall similarities and significant differences in the three-dimensional structures and crystalline properties are discussed for the Sea and Mcg Bence-Jones proteins, which together provide a generalized model of abnormalities present in lambda chains, facilitating a better understanding of amyloidosis of light-chain origin (AL). |
==About this Structure== | ==About this Structure== | ||
| - | 1JVK is a [http://en.wikipedia.org/wiki/Protein_complex Protein complex] structure of sequences from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http:// | + | 1JVK is a [http://en.wikipedia.org/wiki/Protein_complex Protein complex] structure of sequences 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=1JVK OCA]. |
==Reference== | ==Reference== | ||
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[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
[[Category: Protein complex]] | [[Category: Protein complex]] | ||
| - | [[Category: Bourne, P | + | [[Category: Bourne, P C.]] |
| - | [[Category: DeWitt, C | + | [[Category: DeWitt, C R.]] |
| - | [[Category: Edmundson, A | + | [[Category: Edmundson, A B.]] |
| - | [[Category: Fan, Z | + | [[Category: Fan, Z C.]] |
| - | [[Category: Ramsland, P | + | [[Category: Ramsland, P A.]] |
[[Category: Shan, L.]] | [[Category: Shan, L.]] | ||
| - | [[Category: Shultz, B | + | [[Category: Shultz, B B.]] |
| - | [[Category: Terzyan, S | + | [[Category: Terzyan, S S.]] |
[[Category: amyloidogenic]] | [[Category: amyloidogenic]] | ||
[[Category: immunoglobulin light chain dimer]] | [[Category: immunoglobulin light chain dimer]] | ||
| - | ''Page seeded by [http:// | + | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Feb 21 13:27:16 2008'' |
Revision as of 11:27, 21 February 2008
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THREE-DIMENSIONAL STRUCTURE OF AN IMMUNOGLOBULIN LIGHT CHAIN DIMER ACTING AS A LETHAL AMYLOID PRECURSOR
Overview
The X-ray structure of an immunoglobulin light-chain dimer isolated from the urine as a "Bence-Jones protein" from a patient with multiple myeloma and amyloidosis (Sea) was determined at 1.94 A resolution and refined to R and R(free) factors of 0.22 and 0.25, respectively. This "amyloidogenic" protein crystallized in the orthorhombic P2(1)2(1)2(1) space group with unit-cell parameters a = 48.28, b = 83.32, c = 112.59 A as determined at 100 K. In the vital organs (heart and kidneys), the equivalent of the urinary protein produced fibrillar amyloid deposits which were fatal to the patient. Compared with the amyloidogenic Mcg light-chain dimer, the Sea protein was highly soluble in aqueous solutions and only crystallized at concentrations approaching 100 mg ml(-1). Both the Sea and Mcg proteins packed into crystals in highly ordered arrangements typical of strongly diffracting crystals of immunoglobulin fragments. Overall similarities and significant differences in the three-dimensional structures and crystalline properties are discussed for the Sea and Mcg Bence-Jones proteins, which together provide a generalized model of abnormalities present in lambda chains, facilitating a better understanding of amyloidosis of light-chain origin (AL).
About this Structure
1JVK is a Protein complex structure of sequences from Homo sapiens. Full crystallographic information is available from OCA.
Reference
Three-dimensional structure of an immunoglobulin light-chain dimer with amyloidogenic properties., Bourne PC, Ramsland PA, Shan L, Fan ZC, DeWitt CR, Shultz BB, Terzyan SS, Moomaw CR, Slaughter CA, Guddat LW, Edmundson AB, Acta Crystallogr D Biol Crystallogr. 2002 May;58(Pt 5):815-23. Epub 2002, Apr 26. PMID:11976493
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