(Q48788268)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:24804307%20AND%20SRC:MED&resulttype=core&format=json

17 March 2020

title

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

1 reference

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17 March 2020

9

1 reference

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17 March 2020

Anthony R. White

12

1 reference

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17 March 2020

Alexandra Grubman

1

1 reference

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17 March 2020

Jonathan D. Cooper

8

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:24804307%20AND%20SRC:MED&resulttype=core&format=json

17 March 2020

author name string

Eveliina Pollari

2

1 reference

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17 March 2020

Clare Duncan

3

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:24804307%20AND%20SRC:MED&resulttype=core&format=json

17 March 2020

Aphrodite Caragounis

4

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:24804307%20AND%20SRC:MED&resulttype=core&format=json

17 March 2020

Tea Blom

5

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:24804307%20AND%20SRC:MED&resulttype=core&format=json

17 March 2020

Irene Volitakis

6

1 reference

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17 March 2020

Andrew Wong

7

1 reference

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17 March 2020

Jari Koistinaho

10

1 reference

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17 March 2020

Anu Jalanko

11

1 reference

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17 March 2020

Katja M Kanninen

13

1 reference

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17 March 2020

publication date

1 April 2014

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17 March 2020

1 reference

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17 March 2020

volume

6

1 reference

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17 March 2020

issue

4

1 reference

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17 March 2020

page(s)

932-943

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:24804307%20AND%20SRC:MED&resulttype=core&format=json

Lysosomal storage diseases--the horizon expands

17 March 2020

cites work

1 reference

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Mutations in the palmitoyl protein thioesterase gene causing infantile neuronal ceroid lipofuscinosis

21 January 2018

1 reference

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Mice with Ppt1Deltaex4 mutation replicate the INCL phenotype and show an inflammation-associated loss of interneurons

21 January 2018

1 reference

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Disruption of PPT1 or PPT2 causes neuronal ceroid lipofuscinosis in knockout mice

21 January 2018

1 reference

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Thalamocortical neuron loss and localized astrocytosis in the Cln3Deltaex7/8 knock-in mouse model of Batten disease.

21 January 2018

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Osmotic stress changes the expression and subcellular localization of the Batten disease protein CLN3.

21 January 2018

1 reference

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Targeted disruption of the Cln3 gene provides a mouse model for Batten disease. The Batten Mouse Model Consortium [corrected]

21 January 2018

1 reference

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Cerebellar defects in a mouse model of juvenile neuronal ceroid lipofuscinosis.

21 January 2018

1 reference

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The mouse ortholog of the neuronal ceroid lipofuscinosis CLN5 gene encodes a soluble lysosomal glycoprotein expressed in the developing brain

21 January 2018

1 reference

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CLN5 and CLN8 protein association with ceramide synthase: biochemical and proteomic approaches.

21 January 2018

1 reference

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A mouse model for Finnish variant late infantile neuronal ceroid lipofuscinosis, CLN5, reveals neuropathology associated with early aging

21 January 2018

1 reference

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Neuronal ceroid lipofuscinoses

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

Cell biology and function of neuronal ceroid lipofuscinosis-related proteins

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

Glial activation spreads from specific cerebral foci and precedes neurodegeneration in presymptomatic ovine neuronal ceroid lipofuscinosis (CLN6).

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

Palmitoyl protein thioesterase 1 (Ppt1)-deficient mouse neurons show alterations in cholesterol metabolism and calcium homeostasis prior to synaptic dysfunction.

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

CLN3P, the Batten disease protein, localizes to membrane lipid rafts (detergent-resistant membranes).

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

Differential regulation of ceramide in lipid-rich microdomains (rafts): antagonistic role of palmitoyl:protein thioesterase and neutral sphingomyelinase 2

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

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

21 January 2018

1 reference

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Metal ion physiopathology in neurodegenerative disorders

21 January 2018

1 reference

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Counting the zinc-proteins encoded in the human genome

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

Twenty years of metallo-neurobiology: where to now?

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

Increasing Cu bioavailability inhibits Abeta oligomers and tau phosphorylation

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

PBT2 rapidly improves cognition in Alzheimer's Disease: additional phase II analyses

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

Diacetylbis(N(4)-methylthiosemicarbazonato) copper(II) (CuII(atsm)) protects against peroxynitrite-induced nitrosative damage and prolongs survival in amyotrophic lateral sclerosis mouse model

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

Increased zinc and manganese in parallel with neurodegeneration, synaptic protein changes and activation of Akt/GSK3 signaling in ovine CLN6 neuronal ceroid lipofuscinosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

Dual nanomolar and picomolar Zn(II) binding properties of metallothionein

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

Mechanism and regulation of cellular zinc transport

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

Altered levels of high-energy phosphate compounds in fibroblasts from different forms of neuronal ceroid lipofuscinoses: further evidence for mitochondrial involvement

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

Batten disease (JNCL) is linked to disturbances in mitochondrial, cytoskeletal, and synaptic compartments.

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

Distinct early molecular responses to mutations causing vLINCL and JNCL presage ATP synthase subunit C accumulation in cerebellar cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

Mutation of the parkinsonism gene ATP13A2 causes neuronal ceroid-lipofuscinosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

A one base pair deletion in the canine ATP13A2 gene causes exon skipping and late-onset neuronal ceroid lipofuscinosis in the Tibetan terrier

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

A homozygous mutation in KCTD7 links neuronal ceroid lipofuscinosis to the ubiquitin-proteasome system.

21 January 2018

1 reference

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Zinc and 4-hydroxy-2-nonenal mediate lysosomal membrane permeabilization induced by H2O2 in cultured hippocampal neurons.

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

Preferential Zn2+ influx through Ca2+-permeable AMPA/kainate channels triggers prolonged mitochondrial superoxide production

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

Cognitive loss in zinc transporter-3 knock-out mice: a phenocopy for the synaptic and memory deficits of Alzheimer's disease?

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

Cellular zinc and redox buffering capacity of metallothionein/thionein in health and disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

Synaptic release of zinc from brain slices: factors governing release, imaging, and accurate calculation of concentration

21 January 2018

1 reference

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Slc39a14 gene encodes ZIP14, a metal/bicarbonate symporter: similarities to the ZIP8 transporter

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

Effects of a copper-deficient diet on the biochemistry, neural morphology and behavior of aged mice

21 January 2018

1 reference

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

Lysosomal involvement in hepatocyte cytotoxicity induced by Cu(2+) but not Cd(2+).

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

Iron is a sensitive biomarker for inflammation in multiple sclerosis lesions

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

Brain transcriptome perturbations in the Hfe(-/-) mouse model of genetic iron loading.

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

Manganese potentiates in vitro production of proinflammatory cytokines and nitric oxide by microglia through a nuclear factor kappa B-dependent mechanism

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

Uptake of cobalt from the nasal mucosa into the brain via olfactory pathways in rats

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

Mitochondrial DNA damage and a hypoxic response are induced by CoCl(2) in rat neuronal PC12 cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

Metallothionein-I+II in neuroprotection

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

Different redox states of metallothionein/thionein in biological tissue

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

Zinc released from metallothionein-iii may contribute to hippocampal CA1 and thalamic neuronal death following acute brain injury

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

trans repression of the human metallothionein IIA gene promoter by PZ120, a novel 120-kilodalton zinc finger protein

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

Cell-penetrating metal complex optical probes: targeted and responsive systems based on lanthanide luminescence

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

Regional brain atrophy in mouse models of neuronal ceroid lipofuscinosis: a new rostrocaudal perspective

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

Retinal pathology and function in a Cln3 knockout mouse model of juvenile Neuronal Ceroid Lipofuscinosis (batten disease).

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

Neuronal ceroid lipofuscinosis (nclf), a new disorder of the mouse linked to chromosome 9

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C

Toxic effects of cobalt in primary cultures of mouse astrocytes

21 January 2018

1 reference

https://api.crossref.org/works/10.1039%2FC4MT00032C