Elevation of fast but not slow troponin I in the circulation of patients with Becker and Duchenne muscular dystrophy
Autor: | Dan Cox, Eric P. Hoffman, Alan J Russell, Michael Ziemba, Benjamin L. Barthel, Volker Straub, Marissa L Barbieri |
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Rok vydání: | 2021 |
Předmět: |
musculoskeletal diseases
0301 basic medicine Adult Male medicine.medical_specialty Adolescent Physiology Duchenne muscular dystrophy 030105 genetics & heredity 03 medical and health sciences Cellular and Molecular Neuroscience Young Adult 0302 clinical medicine Physiology (medical) Internal medicine Troponin I Myosin medicine Humans Muscular dystrophy Myopathy Child Retrospective Studies biology business.industry Skeletal muscle Middle Aged medicine.disease Troponin Muscular Dystrophy Duchenne medicine.anatomical_structure Endocrinology Cross-Sectional Studies biology.protein Creatine kinase Female Neurology (clinical) medicine.symptom business 030217 neurology & neurosurgery Biomarkers |
Zdroj: | Musclenerve. 64(1) |
ISSN: | 1097-4598 |
Popis: | INTRODUCTION One of the hallmarks of injured skeletal muscle is the appearance of elevated skeletal muscle proteins in circulation. Human skeletal muscle generally consists of a mosaic of slow (type I) and fast (type IIa, IIx/d) fibers, defined by their myosin isoform expression. Recently, measurement of circulating fiber-type specific isoforms of troponin I has been used as a biomarker to suggest that muscle injury in healthy volunteers (HV) results in the appearance of muscle proteins from fast but not slow fibers. We sought to understand if this is also the case in severe myopathy patients with Becker and Duchenne muscular dystrophy (BMD, DMD). METHODS An enzyme-linked immunosorbent assay (ELISA) that selectively measures fast and slow skeletal troponin I (TNNI2 and TNNI1) was used to measure a cross-section of patient plasma samples from HV (N = 50), BMD (N = 49), and DMD (N = 132) patients. Creatine kinase (CK) activity was also measured from the same samples for comparison. RESULTS TNNI2 was elevated in BMD and DMD and correlated with the injury biomarker, CK. In contrast, TNNI1 levels were indistinguishable from levels in HV. There was an inverse relationship between CK and TNNI2 levels and age, but no relationship for TNNI1. DISCUSSION We define a surprising discrepancy between TNNI1 and TNNI2 in patient plasma that may have implications for the interpretation of elevated muscle protein levels in dystrophinopathies. |
Databáze: | OpenAIRE |
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