HARNESSING THE CONSEQUENCES OF IMPAIRED MITOCHONDRIAL ENERGY METABOLISM TO TREAT THE SYMPTOMS OF AGE-RELATED NEURONAL DECLINE

Differential protein expression due to Se deficiency and Se toxicity in rat liver

Barbara Rita Cardoso, Larissa Lago, Aimee Linda Dordevic, Eugene Anthony Kapp, Anna M Raines, Roger A Sunde, Blaine Russel Roberts

2021-08-22

There is a U-shaped dose-response between selenium (Se) status and health outcomes, but underlying metabolic processes are unclear..

Cadmium stress dictates central carbon flux and alters membrane composition in Streptococcus pneumoniae

Stephanie L Neville, Bart A Eijkelkamp, Amber Lothian, James C Paton, Blaine R Roberts, Jason W Rosch, Christopher A McDevitt

2020-12-19

Metal ion homeostasis is essential for all forms of life. However, the breadth of intracellular impacts that arise upon dysregulat..

Choice of mobile phase: Implications for size exclusion chromatography-inductively coupled plasma-mass spectrometry analyses of copper, zinc and iron metalloproteins

Larissa Lago, Oliver RB Thomas, Blaine R Roberts

2020-04-12

The correct identification of the metalloproteins present in human tissues and fluids is essential to our understanding of the cel..

Expanding beyond ICP-MS to better understand selenium biochemistry

Barbara R Cardoso, Katherine Ganio, Blaine R Roberts

2019-12-01

Selenium is an essential trace element in human health and therefore its concentration in biological samples (biofluids and tissue..

Copper Isotope Compositions of Superoxide Dismutase and Metallothionein from Post-Mortem Human Frontal Cortex

Fiona Larner, Catriona A McLean, Alex N Halliday, Blaine R Roberts

2019-07-01

The natural copper isotopic compositions of superoxide dismutase and metallothionein from six post-mortem human frontal cortices w..

Anatomical redistribution of endogenous copper in embryonic mice overexpressing SOD1

K Kysenius, JB Hilton, B Paul, DJ Hare, PJ Crouch

2019-01-01

Mutations in the copper (Cu)- and zinc (Zn)-binding metalloenzyme Cu/Zn-superoxide dismutase (SOD1) cause familial forms of amyotr..

The accumulation of enzymatically inactive cuproenzymes is a CNS-specific phenomenon of the SOD1(G37R) mouse model of ALS and can be restored by overexpressing the human copper transporter hCTR1

James B Hilton, Kai Kysenius, Anthony R White, Peter J Crouch

2018-09-01

Mutations to the copper-dependent enzyme Cu/Zn-superoxide dismutase (SOD1) cause amyotrophic lateral sclerosis (ALS) in humans, an..

Cu-II(atsm) improves the neurological phenotype and survival of SOD1(G93A) mice and selectively increases enzymatically active SOD1 in the spinal cord

James B Hilton, Stephen W Mercer, Nastasia KH Lim, Noel G Faux, Gojko Buncic, Joseph S Beckman, Blaine R Roberts, Paul S Donnelly, Anthony R White, Peter J Crouch

2017-02-13

Ubiquitous expression of mutant Cu/Zn-superoxide dismutase (SOD1) selectively affects motor neurons in the central nervous system ..

Laser ablation-inductively coupled plasma-mass spectrometry imaging of white and gray matter iron distribution in Alzheimer's disease frontal cortex

Dominic J Hare, Erika P Raven, Blaine R Roberts, Mirjana Bogeski, Stuart D Portbury, Catriona A McLean, Colin L Masters, James R Connor, Ashley I Bush, Peter J Crouch, Philip A Doble

2016-08-15

Iron deposition in the brain is a feature of normal aging, though in several neurodegenerative disorders, including Alzheimer's di..

Copper delivery to the CNS by CuATSM effectively treats motor neuron disease in SODG93A mice co-expressing the Copper-Chaperone-for-SOD

Jared R Williams, Emiliano Trias, Pamela R Beilby, Nathan I Lopez, Edwin M Labut, C Samuel Bradford, Blaine R Roberts, Erin J McAllum, Peter J Crouch, Timothy W Rhoads, Cliff Pereira, Marjatta Son, Jeffrey L Elliott, Maria Clara Franco, Alvaro G Estevez, Luis Barbeito, Joseph S Beckman

2016-05-01

Over-expression of mutant copper, zinc superoxide dismutase (SOD) in mice induces ALS and has become the most widely used model of..

Prion protein "gamma-cleavage": characterizing a novel endoproteolytic processing event

Victoria Lewis, Vanessa A Johanssen, Peter J Crouch, Genevieve M Klug, Nigel M Hooper, Steven J Collins

2016-02-01

The cellular prion protein (PrP(C)) is a ubiquitously expressed protein of currently unresolved but potentially diverse function. ..

Endogenous Cu in the central nervous system fails to satiate the elevated requirement for Cu in a mutant SOD1 mouse model of ALS

JB Hilton, AR White, PJ Crouch

2016-01-01

Amyotrophic lateral sclerosis (ALS) is the most common form of motor neuron disease, a fatal degenerative disorder in which motor ..

Zn-II(atsm) is protective in amyotrophic lateral sclerosis model mice via a copper delivery mechanism

Erin J McAllum, Blaine R Roberts, James L Hickey, Theresa N Dang, Alexandra Grubman, Paul S Donnelly, Jeffrey R Liddell, Anthony R White, Peter J Crouch

2015-09-01

Mutations in the metalloprotein Cu,Zn-superoxide dismutase (SOD1) cause approximately 20% of familial cases of amyotrophic lateral..

Oral Treatment with Cu-II(atsm) Increases Mutant SOD1 In Vivo but Protects Motor Neurons and Improves the Phenotype of a Transgenic Mouse Model of Amyotrophic Lateral Sclerosis

Blaine R Roberts, Nastasia KH Lim, Erin J McAllum, Paul S Donnelly, Dominic J Hare, Philip A Doble, Bradley J Turner, Katherine A Price, Sin Chun Lim, Brett M Paterson, James L Hickey, Timothy W Rhoads, Jared R Williams, Katja M Kanninen, Lin W Hung, Jeffrey R Liddell, Alexandra Grubman, Jean-Francois Monty, Roxana M Llanos, David R Kramer

2014-06-04

Mutations in the metallo-protein Cu/Zn-superoxide dismutase (SOD1) cause amyotrophic lateral sclerosis (ALS) in humans and an expr..