Nirma Perera

Amyotrophic Lateral Sclerosis and Autophagy: Dysfunction and Therapeutic Targeting

Azin Amin, Nirma D Perera, Philip M Beart, Bradley J Turner, Fazel Shabanpoor

Journal article  |  2020  |  CELLS

Over the past 20 years, there has been a drastically increased understanding of the genetic basis of Amyotrophic Lateral Sclerosis..

Necroptosis is dispensable for motor neuron degeneration in a mouse model of ALS

Taide Wang, Nirma D Perera, Mathew DF Chiam, Brittany Cuic, Nayomi Wanniarachchillage, Doris Tomas, Andre L Samson, Wayne Cawthorne, Eric N Valor, James M Murphy, Bradley J Turner

Journal article  |  2020  |  CELL DEATH AND DIFFERENTIATION

Motor neuron degeneration in amyotrophic lateral sclerosis (ALS) is proposed to occur by necroptosis, an inflammatory form of regu..

Association of Regulatory T-Cell Expansion With Progression of Amyotrophic Lateral Sclerosis A Study of Humans and a Transgenic Mouse Model

Rebecca K Sheean, Fiona C McKay, Erika Cretney, Christopher R Bye, Nirma D Perera, Doris Tomas, Richard A Weston, Karlene J Scheller, Elvan Djouma, Parvathi Menon, Stephen D Schibeci, Najwa Marmash, Justin J Yerbury, Stephen L Nutt, David R Booth, Graeme J Stewart, Mathew C Kiernan, Steve Vucic, Bradley J Turner

Journal article  |  2018  |  JAMA NEUROLOGY

Importance: Neuroinflammation appears to be a key modulator of disease progression in amyotrophic lateral sclerosis (ALS) and ther..

Rilmenidine promotes MTOR-independent autophagy in the mutant SOD1 mouse model of amyotrophic lateral sclerosis without slowing disease progression

Nirma D Perera, Rebecca K Sheean, Chew L Lau, Yea Seul Shin, Philip M Beart, Malcolm K Horne, Bradley J Turner

Journal article  |  2018  |  AUTOPHAGY

Macroautophagy/autophagy is the main intracellular catabolic pathway in neurons that eliminates misfolded proteins, aggregates and..

TDP-43 mutations causing amyotrophic lateral sclerosis are associated with altered expression of RNA-binding protein hnRNP K and affect the Nrf2 antioxidant pathway

Diane Moujalled, Alexandra Grubman, Karla Acevedo, Shu Yang, Yazi D Ke, Donia M Moujalled, Clare Duncan, Aphrodite Caragounis, Nirma D Perera, Bradley J Turner, Mercedes Prudencio, Leonard Petrucelli, Ian Blair, Lars M Ittner, Peter J Crouch, Jeffrey R Liddell, Anthony R White

Journal article  |  2017  |  HUMAN MOLECULAR GENETICS

TAR DNA binding protein 43 (TDP-43) is a major disease-associated protein involved in the pathogenesis of amyotrophic lateral scle..

Enhancing survival motor neuron expression extends lifespan and attenuates neurodegeneration in mutant TDP-43 mice

Nirma D Perera, Rebecca K Sheean, Peter J Crouch, Anthony R White, Malcolm K Horne, Bradley J Turner

Journal article  |  2016  |  HUMAN MOLECULAR GENETICS

Defects in the RNA-binding proteins survival motor neuron (SMN) and TAR DNA-binding protein 43 (TDP-43) cause progressive motor ne..

Synaptopathies: synaptic dysfunction in neurological disorders - A review from students to students

Katarzyna Lepeta, Mychael V Lourenco, Barbara C Schweitzer, Pamela V Martino Adami, Priyanjalee Banerjee, Silvina Catuara-Solarz, Mario de La Fuente Revenga, Alain Marc Guillem, Mouna Haidar, Omamuyovwi M Ijomone, Bettina Nadorp, Lin Qi, Nirma D Perera, Louise K Refsgaard, Kimberley M Reid, Mariam Sabbar, Arghyadip Sahoo, Natascha Schaefer, Rebecca K Sheean, Anna Suska, et al.

Journal article  |  2016  |  JOURNAL OF NEUROCHEMISTRY

Synapses are essential components of neurons and allow information to travel coordinately throughout the nervous system to adjust ..

AMPK Signalling and Defective Energy Metabolism in Amyotrophic Lateral Sclerosis

Nirma D Perera, Bradley J Turner

Journal article  |  2016  |  NEUROCHEMICAL RESEARCH

Amyotrophic lateral sclerosis (ALS) is caused by selective loss of upper and lower motor neurons by complex mechanisms that are in..

Effect of thymic stimulation of CD4 T cell expansion on disease onset and progression in mutant SOD1 mice

Rebecca K Sheean, Richard H Weston, Nirma D Perera, Angela D'Amico, Stephen L Nutt, Bradley J Turner

Journal article  |  2015  |  JOURNAL OF NEUROINFLAMMATION

BACKGROUND: The peripheral immune system is implicated in modulating microglial activation, neurodegeneration and disease progress..