Journal article
Heterogeneous nuclear ribonucleoproteins in amyotrophic lateral sclerosis: What do we know?
D Moujalled, AR White
Future Neurology | FUTURE MEDICINE LTD | Published : 2014
DOI: 10.2217/fnl.14.7
Abstract
Amyotrophic lateral sclerosis (ALS) is a fatal adult-onset motor neuron disease that results from the progressive loss of motor neurons in the brainstem and spinal cord, and of upper motor neurons in the motor cortex. TDP-43 was the first protein identified in ALS. It is present in cytoplasmic inclusions in motor neurons of affected patient brains and spinal cords, a hallmark feature of this disease. Successive studies have identified missense mutations in TARDBP, and, to date, more than 40 mutations have been identified. Recent studies have indicated that altered RNA metabolism is a key feature of ALS. This article reviews an emerging role of heterogeneous nuclear ribonucleoproteins driving..
View full abstractGrants
Funding Acknowledgements
D Moujalled is supported by funding from the Motor Neuron Disease Research Institute of Australia (MNDRIA). She is also funded by an Alzheimer's Australia Dementia Research fellowship. AR White is supported by an Australian Research Council Future Fellowship and The National Health and Medical Research Council of Australia. Patent protection has previously been sought by the University of Melbourne for the use of bis(thiosemicarbazones) for treatment of diseases. AR White is a coinventor on the patent application PCT/AU2007/001792 that is the subject of a commercialization contract between the University and a private company. The company has not funded nor contributed to research described in this manuscript. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.