Bradley Turner

Altered Blood-Brain Barrier Dynamics in the C9orf72 Hexanucleotide Repeat Expansion Mouse Model of Amyotrophic Lateral Sclerosis

Yijun Pan, Yoshiteru Kagawa, Jiaqi Sun, Bradley J Turner, Cheng Huang, Anup D Shah, Ralf B Schittenhelm, Joseph A Nicolazzo

Journal article  |  2022  |  PHARMACEUTICS

For peripherally administered drugs to reach the central nervous system (CNS) and treat amyotrophic lateral sclerosis (ALS), they ..

Metabolic Dysfunction in Motor Neuron Disease: Shedding Light through the Lens of Autophagy

Subhavi De Silva, Bradley J Turner, Nirma D Perera

Journal article  |  2022  |  METABOLITES

Amyotrophic lateral sclerosis (ALS) patients show a myriad of energetic abnormalities, such as weight loss, hypermetabolism, and d..

Lipid Metabolism Is Dysregulated in the Motor Cortex White Matter in Amyotrophic Lateral Sclerosis

Gemma L Sadler, Katherine N Lewis, Vinod K Narayana, David P De Souza, Joel Mason, Catriona McLean, David G Gonsalvez, Bradley J Turner, Samantha K Barton

Journal article  |  2022  |  METABOLITES

Lipid metabolism is profoundly dysregulated in amyotrophic lateral sclerosis (ALS), yet the lipid composition of the white matter,..

The Amyotrophic Lateral Sclerosis M114T PFN1 Mutation Deregulates Alternative Autophagy Pathways and Mitochondrial Homeostasis

Elisa Teyssou, Laura Chartier, Delphine Roussel, Nirma D Perera, Ivan Nemazanyy, Dominique Langui, Melanie Albert, Thierry Larmonier, Safaa Saker, Francois Salachas, Pierre-Francois Pradat, Vincent Meininger, Philippe Ravassard, Francine Cote, Christian S Lobsiger, Severine Boillee, Bradley J Turner, Danielle Seilhean, Stephanie Millecamps

Journal article  |  2022  |  INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Mutations in profilin 1 (PFN1) have been identified in rare familial cases of Amyotrophic Lateral Sclerosis (ALS). PFN1 is involve..

alpha-Amino-3-hydroxyl-5-methyl-4-isoxazole-propionate receptor and RNA processing gene dysregulation are early determinants of selective motor neuron vulnerability in a mouse model of amyotrophic lateral sclerosis

Pardis F Zanganeh, Samantha K Barton, Katherine Lim, Elizabeth L Qian, Duncan E Crombie, Christopher R Bye, Bradley J Turner

Journal article  |  2022  |  BRAIN COMMUNICATIONS

Amyotrophic lateral sclerosis is a late-onset adult neurodegenerative disease, although there is mounting electrophysiological and..

Mapping Motor Neuron Vulnerability in the Neuraxis of Male SOD1(G93A) Mice Reveals Widespread Loss of Androgen Receptor Occurring Early in Spinal Motor Neurons

Victoria M McLeod, Mathew DF Chiam, Nirma D Perera, Chew L Lau, Wah Chin Boon, Bradley J Turner

Journal article  |  2022  |  FRONTIERS IN ENDOCRINOLOGY

Sex steroid hormones have been implicated as disease modifiers in the neurodegenerative disorder amyotrophic lateral sclerosis (AL..

Functional characterisation of the amyotrophic lateral sclerosis risk locus GPX3/TNIP1

Restuadi Restuadi, Frederik J Steyn, Edor Kabashi, Shyuan T Ngo, Fei-Fei Cheng, Marta F Nabais, Mike J Thompson, Ting Qi, Yang Wu, Anjali K Henders, Leanne Wallace, Chris R Bye, Bradley J Turner, Laura Ziser, Susan Mathers, Pamela A McCombe, Merrilee Needham, David Schultz, Matthew C Kiernan, Wouter van Rheenen, et al.

Journal article  |  2022  |  GENOME MEDICINE

BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a complex, late-onset, neurodegenerative disease with a genetic contribution to..

Ferroptosis mediates selective motor neuron death in amyotrophic lateral sclerosis

Taide Wang, Doris Tomas, Nirma D Perera, Brittany Cuic, Sophia Luikinga, Aida Viden, Samantha K Barton, Catriona A McLean, Andre L Samson, Adam Southon, Ashley I Bush, James M Murphy, Bradley J Turner

Journal article  |  2021  |  CELL DEATH AND DIFFERENTIATION

Amyotrophic lateral sclerosis (ALS) is caused by selective degeneration of motor neurons in the brain and spinal cord; however, th..

Look youse guys and gals, dat just ain't right

Daniel J Klionsky

Journal article  |  2021  |  AUTOPHAGY

In 2008, we published the first set of guidelines for standardizing research in autophagy. Since then, this topic has received inc..

Advances in Gene Delivery Methods to Label and Modulate Activity of Upper Motor Neurons: Implications for Amyotrophic Lateral Sclerosis

Mouna Haidar, Aida Viden, Bradley J Turner

Journal article  |  2021  |  BRAIN SCIENCES

The selective degeneration of both upper motor neurons (UMNs) and lower motor neurons (LMNs) is the pathological hallmark of amyot..