Huber, Robert J.
Published in
Journal of Biomedical Science
The neuronal ceroid lipofuscinoses (NCLs), commonly known as Batten disease, belong to a family of neurological disorders that cause blindness, seizures, loss of motor function and cognitive ability, and premature death. There are 13 different subtypes of NCL that are associated with mutations in 13 genetically distinct genes (CLN1-CLN8, CLN10-CLN1...
Winter, Aimee N. Subbarayan, Meena S. Grimmig, Bethany Weesner, Jason A. Moss, Lauren Peters, Melinda Weeber, Edwin Nash, Kevin Bickford, Paula C.
Published in
Journal of Neuroinflammation
BackgroundFractalkine (CX3CL1; FKN) is a chemokine expressed by neurons that mediates communication between neurons and microglia. By regulating microglial activity, CX3CL1 can mitigate the damaging effects of chronic microglial inflammation within the brain, a state that plays a major role in aging and neurodegeneration. CX3CL1 is present in two f...
Bettencourt, Conceição Piras, Ignazio S. Foti, Sandrine C. Talboom, Joshua Miki, Yasuo Lashley, Tammaryn Balazs, Robert Viré, Emmanuelle Warner, Thomas T. Huentelman, Matt J.
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Published in
Acta Neuropathologica Communications
James, Rachel E. Schalks, Renee Browne, Eleanor Eleftheriadou, Ioanna Munoz, Carmen Picon Mazarakis, Nicholas D. Reynolds, Richard
Published in
Acta Neuropathologica Communications
Analysis of isolated meninges and cerebrospinal fluid (CSF) of post-mortem MS cases has shown increased gene and protein expression for the pro-inflammatory cytokines: tumour necrosis factor (TNF) and interferon-γ (IFNγ). Here we tested the hypothesis that persistent production of these cytokines in the meningeal compartment and diffusion into unde...
Gravesteijn, A. S. Beckerman, H. de Jong, B. A. Hulst, H. E. de Groot, V.
Published in
BMC Neurology
BackgroundNeurodegeneration, rather than inflammation, plays a key role in the progressive phase of multiple sclerosis (MS). Current disease modifying treatment options for people with progressive MS (PMS) do not specifically target neurodegeneration. Preliminary evidence suggests that exercise therapy might have neuroprotective effects. However, n...
Tan, Meng-Shan Ji, Xi Li, Jie-Qiong Xu, Wei Wang, Hui-Fu Tan, Chen-Chen Dong, Qiang Zuo, Chuan-Tao Tan, Lan Suckling, John
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Published in
Alzheimer's Research & Therapy
BackgroundModels of Alzheimer’s disease (AD) pathophysiology posit that amyloidosis [A] precedes and accelerates tau pathology [T] that leads to neurodegeneration [N]. Besides this A-T-N sequence, other biomarker sequences are possible. This current work investigates and compares the longitudinal trajectories of Alzheimer’s ATN biomarker profiles i...
Song, Qiaoyun Meng, Bo Xu, Haidong Mao, Zixu
Published in
Translational Neurodegeneration
BackgroundLysosomes digest extracellular material from the endocytic pathway and intracellular material from the autophagic pathway. This process is performed by the resident hydrolytic enzymes activated by the highly acidic pH within the lysosomal lumen. Lysosome pH gradients are mainly maintained by the vacuolar (H+) ATPase (or V-ATPase), which p...
Gallart-Palau, Xavier Guo, Xue Serra, Aida Sze, Siu Kwan
Published in
Alzheimer's Research & Therapy
BackgroundThe contributions of brain intercellular communication mechanisms, specifically extracellular vesicles (EV), to the progression of Alzheimer’s disease (AD) remain poorly understood.MethodsHere, we investigated the role(s) of brain EV in the progressive course of AD through unbiased proteome-wide analyses of temporal lobe-derived EV and pr...
Carroll, James A. Race, Brent Williams, Katie Striebel, James Chesebro, Bruce
Published in
Molecular Brain
BackgroundPrion diseases and prion-like disorders, including Alzheimer’s disease and Parkinson’s disease, are characterized by gliosis and accumulation of misfolded aggregated host proteins. Ablating microglia in prion-infected brain by treatment with the colony-stimulating factor-1 receptor (CSF-1R) inhibitor, PLX5622, increased accumulation of mi...
Anilkumar, Ujval Khacho, Mireille Cuillerier, Alexanne Harris, Richard Patten, David A. Bilen, Maria Iqbal, Mohamed Ariff Guo, Ding Yuan Trudeau, Louis-Eric Park, David S.
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Published in
Cell Death & Disease
Mitochondria play a crucial role in neuronal survival through efficient energy metabolism. In pathological conditions, mitochondrial stress leads to neuronal death, which is regulated by the anti-apoptotic BCL-2 family of proteins. MCL-1 is an anti-apoptotic BCL-2 protein localized to mitochondria either in the outer membrane (OM) or inner membrane...
