Deregulation of biometal homeostasis: the missing link for neuronal ceroid lipofuscinoses?

PBT2 Rapidly Improves Cognition in Alzheimer's Disease: Additional Phase II Analyses

(2017) Noel G. Faux et al.   JOURNAL OF ALZHEIMERS DISEASE

Deregulation of subcellular biometal homeostasis through loss of the metal transporter, Zip7, in a childhood neurodegenerative disorder

(2014) Alexandra Grubman et al.   Acta Neuropathologica Communications

Cell biology and function of neuronal ceroid lipofuscinosis-related proteins

(2013) Katrin Kollmann et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Regional Brain Atrophy in Mouse Models of Neuronal Ceroid Lipofuscinosis

(2013) Thomas G. Kühl et al.   JOURNAL OF CHILD NEUROLOGY

Inflammation alters the expression of DMT1, FPN1 and hepcidin, and it causes iron accumulation in central nervous system cells

(2013) Pamela Urrutia et al.   JOURNAL OF NEUROCHEMISTRY

Lysosomal storage diseases—the horizon expands

(2013) Rose-Mary Naaman Boustany   Nature Reviews Neurology

Increased Zinc and Manganese in Parallel with Neurodegeneration, Synaptic Protein Changes and Activation of Akt/GSK3 Signaling in Ovine CLN6 Neuronal Ceroid Lipofuscinosis

(2013) Katja M. Kanninen et al.   PLoS One

Iron Is a Sensitive Biomarker for Inflammation in Multiple Sclerosis Lesions

(2013) Veela Mehta et al.   PLoS One

Osmotic Stress Changes the Expression and Subcellular Localization of the Batten Disease Protein CLN3

(2013) Amanda Getty et al.   PLoS One

Manganese neurotoxicity: new perspectives from behavioral, neuroimaging, and neuropathological studies in humans and non-human primates

(2013) Tomás R. Guilarte   Frontiers in Aging Neuroscience

Altered biometal homeostasis is associated with CLN6 mRNA loss in mouse neuronal ceroid lipofuscinosis

(2013) K. M. Kanninen et al.   Biology Open

A Homozygous Mutation in KCTD7 Links Neuronal Ceroid Lipofuscinosis to the Ubiquitin-Proteasome System

(2012) John F. Staropoli et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Brain transcriptome perturbations in the Hfe−/− mouse model of genetic iron loading

(2012) Daniel Johnstone et al.   BRAIN RESEARCH

Exacerbated neuronal ceroid lipofuscinosis phenotype in Cln1/5 double-knockout mice

(2012) T. Blom et al.   Disease Models & Mechanisms

CLN5 and CLN8 protein association with ceramide synthase: Biochemical and proteomic approaches

(2012) Saria El Haddad et al.   ELECTROPHORESIS

Mutation of the parkinsonism gene ATP13A2 causes neuronal ceroid-lipofuscinosis

(2012) Jose Bras et al.   HUMAN MOLECULAR GENETICS

The hypoxia imaging agent Cu II (atsm) is neuroprotective and improves motor and cognitive functions in multiple animal models of Parkinson’s disease

(2012) Lin W. Hung et al.   JOURNAL OF EXPERIMENTAL MEDICINE

Effects of a Copper-Deficient Diet on the Biochemistry, Neural Morphology and Behavior of Aged Mice

(2012) Silvia Bolognin et al.   PLoS One

Diacetylbis(N(4)-methylthiosemicarbazonato) Copper(II) (CuII(atsm)) Protects against Peroxynitrite-induced Nitrosative Damage and Prolongs Survival in Amyotrophic Lateral Sclerosis Mouse Model

(2011) Cynthia P. W. Soon et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Subcellular localization of a fluorescent derivative of CuII(atsm) offers insight into the neuroprotective action of CuII(atsm)

(2011) Katherine Ann Price et al.   Metallomics

A truncating mutation in ATP13A2 is responsible for adult-onset neuronal ceroid lipofuscinosis in Tibetan terriers

(2011) Fabiana H.G. Farias et al.   NEUROBIOLOGY OF DISEASE

Cln5-deficiency in mice leads to microglial activation, defective myelination and changes in lipid metabolism

(2011) Mia-Lisa Schmiedt et al.   NEUROBIOLOGY OF DISEASE

Distinct Early Molecular Responses to Mutations Causing vLINCL and JNCL Presage ATP Synthase Subunit C Accumulation in Cerebellar Cells

(2011) Yi Cao et al.   PLoS One

Measuring steady-state and dynamic endoplasmic reticulum and Golgi Zn2+ with genetically encoded sensors

(2011) Y. Qin et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

A One Base Pair Deletion in the Canine ATP13A2 Gene Causes Exon Skipping and Late-Onset Neuronal Ceroid Lipofuscinosis in the Tibetan Terrier

(2011) Anne Wöhlke et al.   PLoS Genetics

Lipofuscin-bound iron is a major intracellular source of oxidants: Role in senescent cells

(2010) Annika Höhn et al.   FREE RADICAL BIOLOGY AND MEDICINE

Expression and lysosomal targeting of CLN7, a major facilitator superfamily transporter associated with variant late-infantile neuronal ceroid lipofuscinosis

(2010) A. Sharifi et al.   HUMAN MOLECULAR GENETICS

Cognitive Loss in Zinc Transporter-3 Knock-Out Mice: A Phenocopy for the Synaptic and Memory Deficits of Alzheimer's Disease?

(2010) P. A. Adlard et al.   JOURNAL OF NEUROSCIENCE

Genome-wide microarray analysis of brain gene expression in mice on a short-term high iron diet

(2010) Daniel Johnstone et al.   NEUROCHEMISTRY INTERNATIONAL

Cell-Penetrating Metal Complex Optical Probes: Targeted and Responsive Systems Based on Lanthanide Luminescence

(2009) Craig P. Montgomery et al.   ACCOUNTS OF CHEMICAL RESEARCH

Metallothionein-I+II in neuroprotection

(2009) Mie Ø. Pedersen et al.   BIOFACTORS

Novel interactions of CLN5 support molecular networking between Neuronal Ceroid Lipofuscinosis proteins

(2009) Annina Lyly et al.   BMC CELL BIOLOGY

Cerebellar defects in a mouse model of juvenile neuronal ceroid lipofuscinosis

(2009) Jill M. Weimer et al.   BRAIN RESEARCH

Progressive thalamocortical neuron loss in Cln5 deficient mice: Distinct effects in Finnish variant late infantile NCL

(2009) Carina von Schantz et al.   NEUROBIOLOGY OF DISEASE

Metal Ion Physiopathology in Neurodegenerative Disorders

(2009) Silvia Bolognin et al.   NEUROMOLECULAR MEDICINE

Increasing Cu bioavailability inhibits A  oligomers and tau phosphorylation

(2009) P. J. Crouch et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The multi-faceted basis of vitamin B12 (cobalamin) neurotrophism in adult central nervous system: Lessons learned from its deficiency

(2009) G. Scalabrino   PROGRESS IN NEUROBIOLOGY

Neuronal ceroid lipofuscinoses

(2008) Anu Jalanko et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Brain gene expression profiles of Cln1 and Cln5 deficient mice unravels common molecular pathways underlying neuronal degeneration in NCL diseases

(2008) Carina von Schantz et al.   BMC GENOMICS

Deficiency of the INCL protein Ppt1 results in changes in ectopic F1-ATP synthase and altered cholesterol metabolism

(2008) Annina Lyly et al.   HUMAN MOLECULAR GENETICS

Accumulation of bis(monoacylglycero)phosphate and gangliosides in mouse models of neuronal ceroid lipofuscinosis

(2008) Sabrina Jabs et al.   JOURNAL OF NEUROCHEMISTRY

Zinc and 4-Hydroxy-2-Nonenal Mediate Lysosomal Membrane Permeabilization Induced by H2O2 in Cultured Hippocampal Neurons

(2008) J. J. Hwang et al.   JOURNAL OF NEUROSCIENCE

Slc39a14 Gene Encodes ZIP14, A Metal/Bicarbonate Symporter: Similarities to the ZIP8 Transporter

(2008) K. Girijashanker et al.   MOLECULAR PHARMACOLOGY