Friedreich's ataxia

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Related to Friedreich ataxia: myotonic dystrophy
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Noun1.Friedreich's ataxia - sclerosis of the posterior and lateral columns of the spinal cord; characterized by muscular weakness and abnormal gait; occurs in children
ataxia, ataxy, dyssynergia, motor ataxia - inability to coordinate voluntary muscle movements; unsteady movements and staggering gait
References in periodicals archive ?
Another two patients were diagnosed as Friedreich ataxia, belonging to hereditary ataxia Typ-I (spinal type).
The report provides a snapshot of the global therapeutic landscape of Friedreich Ataxia
Mouse models for Friedreich ataxia exhibit cardiomyopathy, sensory nerve defect and Fe-S enzyme deficiency followed by intramitochondrial iron deposits.
Alice Pebay and Mirella Dottori, co-leaders of the study from University of Melbourne, characterized and directed the Friedreich ataxia iPS cells to become specific cell types, including heart cells and nerves, which are normally not functioning well in the disease.
Dottori said the research could not have been achieved without a significant network of experts and support from the Friedreich Ataxia Research Association (Australasia) (FARA-A) and the Friedreich Ataxia Research Alliance (FARA) in the United States.
Some disorders related to mitochondrial iron metabolism Disease OMIM# Inheritance Related eene locus Friedreich ataxia 229300 Autosomal 9ql3,9p23-pll recessive X-linked sideroblastic 300751 X- linked XplL2l anemia recessive Pyridoxine-unresponsive 205950 Autosomal 14q32, 3p22.
Autosomal dominant spinocerebellar ataxia (ADSCA) (1,2,3) and Friedreich ataxia (FRDA) (4,5,6) are neurodegenerative and neurometabolic disorders whose characteristics include progressive impairment of balance and motor functions.