نتایج جستجو برای: mitochondrial disorder

تعداد نتایج: 719698  

Journal: :PLoS Genetics 2009
Izabella Baranowska Karin Hultin Jäderlund Inger Nennesmo Erik Holmqvist Nadja Heidrich Nils-Göran Larsson Göran Andersson E. Gerhart H. Wagner Åke Hedhammar Rolf Wibom Leif Andersson

Sensory ataxic neuropathy (SAN) is a recently identified neurological disorder in golden retrievers. Pedigree analysis revealed that all affected dogs belong to one maternal lineage, and a statistical analysis showed that the disorder has a mitochondrial origin. A one base pair deletion in the mitochondrial tRNA(Tyr) gene was identified at position 5304 in affected dogs after re-sequencing the ...

Journal: :International Journal of Developmental Neuroscience 2010

2015
Elizabeth M. McCormick Lawrence Kenyon Marni J. Falk

Desmin (DES) is a major muscle scaffolding protein that also functions to anchor mitochondria. Pathogenic DES mutations, however, have not previously been recognized as a cause of multi-systemic mitochondrial disease. Here, we describe a 45-year-old man who presented to The Children's Hospital of Philadelphia Mitochondrial-Genetics Diagnostic Clinic for evaluation of progressive cardiac, neurom...

Journal: :Journal of Postgraduate Medicine, Education and Research 2012

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