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Mutations in nuclear-encoded protein subunits of the mitochondrial ribosome are an increasingly recognised cause of oxidative phosphorylation system (OXPHOS) disorders. Among them, mutations in the MRPL44 gene, encoding a structural protein of the large subunit of the mitochondrial ribosome, have been identified in four patients with OXPHOS defects and early-onset hypertrophic cardiomyopathy with or without additional clinical features. A 23-year-old individual with cardiac and skeletal myopathy, neurological involvement, and combined deficiency of OXPHOS complexes in skeletal muscle was clinically and genetically investigated. Analysis of whole-exome sequencing data revealed a homozygous mutation in MRPL44 (c.467 T?>?G), which was not present in the biological father, and a region of homozygosity involving most of chromosome 2, raising the possibility of uniparental disomy. Short-tandem repeat and genome-wide SNP microarray analyses of the family trio confirmed complete maternal uniparental isodisomy of chromosome 2. Mitochondrial ribosome assembly and mitochondrial translation were assessed in patient derived-fibroblasts. These studies confirmed that c.467 T?>?G affects the stability or assembly of the large subunit of the mitochondrial ribosome, leading to impaired mitochondrial protein synthesis and decreased levels of multiple OXPHOS components. This study provides evidence of complete maternal uniparental isodisomy of chromosome 2 in a patient with MRPL44-related disease, and confirms that MRLP44 mutations cause a mitochondrial translation defect that may present as a multisystem disorder with neurological involvement.
相似文献Campesinos are the most numerous hunters in Latin America. Yet, local and traditional knowledge (LTK) among campesinos about hunting is often invisible to conservationists who perceive them as nonindigenous or illegal hunters. Moreover, research and methods for accessing campesino hunting LTK are limited in theory and practice. Conservationists therefore know little about campesinos’ cultural understandings of hunting. We assessed the LTK of Nicaraguan campesinos to determine whether they shared cultural hunting knowledge. Through 11 months of ethnographic fieldwork, an ethnoecology framework, and cultural consensus analysis, we found that campesino hunting LTK was shared across the study community. This knowledge extended from a worldview that emphasized subsistence and hunting secrets to ensure bountiful harvests, expressed through folk taxonomies, hunting strategies, campesino-dog relationships, and preparation of hunted animals. Campesino hunting LTK emerged from campesino culture, yielding numerous implications for conservation in Latin America.
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