1plp

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[[Image:1plp.png|left|200px]]
 
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{{STRUCTURE_1plp| PDB=1plp | SCENE= }}
{{STRUCTURE_1plp| PDB=1plp | SCENE= }}
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===SOLUTION STRUCTURE OF THE CYTOPLASMIC DOMAIN OF PHOSPHOLAMBAN===
===SOLUTION STRUCTURE OF THE CYTOPLASMIC DOMAIN OF PHOSPHOLAMBAN===
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{{ABSTRACT_PUBMED_7779806}}
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{{ABSTRACT_PUBMED_7779806}}
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==Disease==
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[[http://www.uniprot.org/uniprot/PPLA_HUMAN PPLA_HUMAN]] Defects in PLN are the cause of cardiomyopathy dilated type 1P (CMD1P) [MIM:[http://omim.org/entry/609909 609909]]. Dilated cardiomyopathy is a disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death.<ref>PMID:12610310</ref><ref>PMID:16432188</ref> Defects in PLN are the cause of familial hypertrophic cardiomyopathy type 18 (CMH18) [MIM:[http://omim.org/entry/613874 613874]]. CMH18 is a hereditary heart disorder characterized by ventricular hypertrophy, which is usually asymmetric and often involves the interventricular septum. The symptoms include dyspnea, syncope, collapse, palpitations, and chest pain. They can be readily provoked by exercise. The disorder has inter- and intrafamilial variability ranging from benign to malignant forms with high risk of cardiac failure and sudden cardiac death.<ref>PMID:12705874</ref>
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==Function==
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[[http://www.uniprot.org/uniprot/PPLA_HUMAN PPLA_HUMAN]] Phospholamban has been postulated to regulate the activity of the calcium pump of cardiac sarcoplasmic reticulum.
==About this Structure==
==About this Structure==
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==Reference==
==Reference==
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<ref group="xtra">PMID:007779806</ref><references group="xtra"/>
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<ref group="xtra">PMID:007779806</ref><references group="xtra"/><references/>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: Burke, C J.]]
[[Category: Burke, C J.]]

Revision as of 10:05, 24 March 2013

Template:STRUCTURE 1plp

Contents

SOLUTION STRUCTURE OF THE CYTOPLASMIC DOMAIN OF PHOSPHOLAMBAN

Template:ABSTRACT PUBMED 7779806

Disease

[PPLA_HUMAN] Defects in PLN are the cause of cardiomyopathy dilated type 1P (CMD1P) [MIM:609909]. Dilated cardiomyopathy is a disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death.[1][2] Defects in PLN are the cause of familial hypertrophic cardiomyopathy type 18 (CMH18) [MIM:613874]. CMH18 is a hereditary heart disorder characterized by ventricular hypertrophy, which is usually asymmetric and often involves the interventricular septum. The symptoms include dyspnea, syncope, collapse, palpitations, and chest pain. They can be readily provoked by exercise. The disorder has inter- and intrafamilial variability ranging from benign to malignant forms with high risk of cardiac failure and sudden cardiac death.[3]

Function

[PPLA_HUMAN] Phospholamban has been postulated to regulate the activity of the calcium pump of cardiac sarcoplasmic reticulum.

About this Structure

1plp is a 1 chain structure with sequence from Homo sapiens. Full experimental information is available from OCA.

Reference

  • Mortishire-Smith RJ, Pitzenberger SM, Burke CJ, Middaugh CR, Garsky VM, Johnson RG. Solution structure of the cytoplasmic domain of phopholamban: phosphorylation leads to a local perturbation in secondary structure. Biochemistry. 1995 Jun 13;34(23):7603-13. PMID:7779806
  1. ↑ Schmitt JP, Kamisago M, Asahi M, Li GH, Ahmad F, Mende U, Kranias EG, MacLennan DH, Seidman JG, Seidman CE. Dilated cardiomyopathy and heart failure caused by a mutation in phospholamban. Science. 2003 Feb 28;299(5611):1410-3. PMID:12610310 doi:10.1126/science.1081578
  2. ↑ Haghighi K, Kolokathis F, Gramolini AO, Waggoner JR, Pater L, Lynch RA, Fan GC, Tsiapras D, Parekh RR, Dorn GW 2nd, MacLennan DH, Kremastinos DT, Kranias EG. A mutation in the human phospholamban gene, deleting arginine 14, results in lethal, hereditary cardiomyopathy. Proc Natl Acad Sci U S A. 2006 Jan 31;103(5):1388-93. Epub 2006 Jan 23. PMID:16432188 doi:10.1073/pnas.0510519103
  3. ↑ Minamisawa S, Sato Y, Tatsuguchi Y, Fujino T, Imamura S, Uetsuka Y, Nakazawa M, Matsuoka R. Mutation of the phospholamban promoter associated with hypertrophic cardiomyopathy. Biochem Biophys Res Commun. 2003 Apr 25;304(1):1-4. PMID:12705874

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