Serum Galectin-1 in Patients with Duchenne Muscular Dystrophy

Abstract

Muscular Dystrophies (MD’s) are a heterogeneous group of degenerative disorders often characterized by progressive muscle weakness and fragility. Many of these diseases result from mutations in genes encoding proteins of the dystrophin-glycoprotein complex (DGC). The most common and severe form among children is Duchenne muscular dystrophy (DMD), caused by mutations in the dystrophin gene, with an average life expectancy around 25 years of age . Galectin-1 has been shown to modulate inflammation and angiogenesis, both critical modifiers of disease progression in DMD. In addition to putative roles of Gal-1 in muscle development, Gal-1 may also mediate several aspects of muscle regeneration. Gal-1 exhibits a protective effect on tissue during damage, possibly through reducing the deleterious sequelae associated with inflammation. Hematopoietic cells expressing CD45 were demonstrated to have myogenic potential during the process of muscle repair Also, hematopoietic cells expressing CD34 is an hematopoietic stem cell enriched population , has recently been associated with both the quiescent and activated states of myogenic satellite cells It was reported that CD34 cells are present in DMD patients for tissue regeneration. It has been demonstrated that CD45 subset comprise juvenile protective factors for the maintenance of brain microvascular health . This study is a cross sectional study and it conducted on 20 patients recruited from Neurology out Patient clinic of Pediatric Hospital, Faculty of Medicine; Ain shams University. The Patients were selected on the basis of the following eligibility criteria: their age was (5-10years). Diagnosis of muscular dystrophy was based on clinical onset of progressive weakness before 5 years of age, and either molecular assessment of mutation in the DMD gene or muscle biopsy that was deficient in dystrophin and compatible with DMD (Hinton et al., 2000). Children with acute pathology who had either cardiac or respiratory insufficiency or children with chronic inflammatory diseses and allergic diseses were excluded. Ten controls age and socioeconomic matching healthy child. They made ordinary blood checkup. All patients included in this study subjected to full history which include (present history,associated side effect,medical histroy,family history) and clinical examination which include general examination and neurological examination.

Blood was drawn on EDTA sterile tubes and the following tests were carried out: 1. Measuring serum creatine phosphokinase (CPK) level 2. 2-Determination of Galactin-1 3. 3-Flow Cytometry Analysis of CD34 and CD45 markers on circulating mononuclear cells.