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

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..

Optimizing red blood cell protein extraction for biomarker quantitation with mass spectrometry

Stephan Klatt, Anne Roberts, Amber Lothian, Colin L Masters, Roberto Cappai, Christopher Fowler, Blaine R Roberts

2020-02-06

Red blood cells (RBC) are the most common cell type found in blood. They might serve as reservoir for biomarker research as they a..

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..