MLP (muscle LIM protein) as a stress sensor in the heart
| Autor: | Keat-Eng Ng, Ching-Hsin Ku, Izabela Piotrowska, Byambajav Buyandelger, Snjezana Miocic, Sylvia Gunkel, Ralph Knöll |
|---|---|
| Rok vydání: | 2011 |
| Předmět: |
Cardiomyopathy
Dilated medicine.medical_specialty Physiology Clinical Biochemistry ved/biology.organism_classification_rank.species Cardiomyopathy Muscle Proteins Cardiomyocyte Biology Mechanotransduction Cellular Mechanosensitivity Mechanoreceptor Physiology (medical) Internal medicine medicine Animals Humans Cardiac muscle CSRP3 Model organism Muscle stretch Invited Review Myogenesis ved/biology Myocardium Cardiac function Heart Dilated cardiomyopathy Cardiomyopathy Hypertrophic LIM Domain Proteins medicine.disease Phenotype Cardiac myocytes Cardiac sarcomere medicine.anatomical_structure Endocrinology Cardiovascular control Heart failure Stress Mechanical Gene expression Neuroscience |
| Zdroj: | Pflugers Archiv |
| ISSN: | 1432-2013 0031-6768 |
| DOI: | 10.1007/s00424-011-0961-2 |
| Popis: | Muscle LIM protein (MLP, also known as cysteine rich protein 3 (CSRP3, CRP3)) is a muscle-specific-expressed LIM-only protein. It consists of 194 amino-acids and has been described initially as a factor involved in myogenesis (Arber et al. Cell 79:221–231, 1994). MLP soon became an important model for experimental cardiology when it was first demonstrated that MLP deficiency leads to myocardial hypertrophy followed by a dilated cardiomyopathy and heart failure phenotype (Arber et al. Cell 88:393–403, 1997). At this time, this was the first genetically altered animal model to develop this devastating disease. Interestingly, MLP was also found to be down-regulated in humans with heart failure (Zolk et al. Circulation 101:2674–2677, 2000) and MLP mutations are able to cause hypertrophic and dilated forms of cardiomyopathy in humans (Bos et al. Mol Genet Metab 88:78–85, 2006; Geier et al. Circulation 107:1390–1395, 2003; Hershberger et al. Clin Transl Sci 1:21–26, 2008; Knöll et al. Cell 111:943–955, 2002; Knöll et al. Circ Res 106:695–704, 2010; Mohapatra et al. Mol Genet Metab 80:207–215, 2003). Although considerable efforts have been undertaken to unravel the underlying molecular mechanisms—how MLP mutations, either in model organisms or in the human setting cause these diseases are still unclear. In contrast, only precise knowledge of the underlying molecular mechanisms will allow the development of novel and innovative therapeutic strategies to combat this otherwise lethal condition. The focus of this review will be on the function of MLP in cardiac mechanosensation and we shall point to possible future directions in MLP research. |
| Databáze: | OpenAIRE |
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