Hypoplastic left heart syndrome myocytes are differentiated but possess a unique phenotype
| Autor: | Shuping Ge, Wayne Minobe, Steve M. Helmke, Michael R. Bristow, M. Benjamin Perryman, Alastair D. Robertson, Gary Brodsky, Teresa J. Bohlmeyer, Jennifer Lynch, James H Sederberg |
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| Rok vydání: | 2003 |
| Předmět: |
Adult
Male CD31 Pathology medicine.medical_specialty Adolescent Blotting Western Connective tissue Biology Polymerase Chain Reaction Mass Spectrometry Pathology and Forensic Medicine Hypoplastic left heart syndrome Hypoplastic Left Heart Syndrome medicine Humans Myocyte Electrophoresis Gel Two-Dimensional Myocytes Cardiac RNA Messenger Child Fetus Gene Expression Profiling Infant Newborn Infant Cell Differentiation General Medicine Cadherins medicine.disease Immunohistochemistry Platelet Endothelial Cell Adhesion Molecule-1 Phenotype medicine.anatomical_structure Child Preschool Heart failure Cardiology and Cardiovascular Medicine Intercalated disc |
| Zdroj: | Cardiovascular Pathology. 12:23-31 |
| ISSN: | 1054-8807 |
| DOI: | 10.1016/s1054-8807(02)00127-8 |
| Popis: | Introduction: Hypoplastic left heart syndrome (HLHS) is the term used to describe a group of congenital malformations characterized by marked underdevelopment of the left side of the heart. HLHS accounts for nearly 25% of cardiac deaths in the first year of life. Although much has been reported regarding diagnosis, gross morphology and surgical treatment, no information on gene expression in HLHS myocytes is available. Methods: We examined heart tissue from patients with HLHS using routine histology, immunohistochemistry, quantitative polymerase chain reaction (PCR), two-dimensional (2-D) gel electrophoresis and protein identification by mass spectrometry. Results: Histologic examination of right and left ventricles from HLHS patients revealed characteristic features of myocyte differentiation, including striations and intercalated disc formation. Immunohistochemical staining using antibody to N-cadherin demonstrated clear development of intercalated discs between myocytes. However, many of the myocytes contained scant cytoplasm and were grouped in small, disorganized bundles separated by abundant connective tissue and dilated, thin-walled vessels. Quantitative PCR analysis demonstrated that both left and right ventricular tissue from HLHS hearts expressed the fetal or “heart failure” gene expression pattern. Two-dimensional gel electrophoresis and protein identification by mass spectrometry also confirmed that myocytes from HLHS ventricles were differentiated but expressed the fetal isoform of some cardiac specific proteins. However, HLHS myocytes in all of the heart samples (n=21) were inappropriately expressing platelet-endothelial cell adhesion molecule-1 (PECAM-1, CD31), a member of the cell adhesion molecule (CAM) family that has a primary role in the regulation of tissue morphogenesis. These findings indicate that myocytes from HLHS syndrome patients, while differentiated, have a unique gene expression pattern. |
| Databáze: | OpenAIRE |
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