ArticleAnnals of neurology2014
Metallothioneins as dynamic markers for brain disease in lysosomal disorders.
Article in Annals of neurology, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers, 1 of them a synthesis that pooled it.
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Who cites it
13 citing papers in PubMed, 1 synthesis or guideline pooled it, 20 citations in OpenAlex.
- Unveiling sex-based differences in Parkinson's disease: a comprehensive meta-analysis of transcriptomic studies.Biology of sex differences · 2022Pooled it
- GlycoRNA-rich, neutrophil membrane-coated, siMT1-loaded nanoparticles mitigate abdominal aortic aneurysm progression by inhibiting the formation of neutrophil extracellular traps.Materials today. Bio · 2025Article
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- AnMolecular genetics and metabolism reports · 2023Article
- Human iPSC-derived astrocytes generated from donors with globoid cell leukodystrophy display phenotypes associated with disease.PloS one · 2022Article
- Metallothionein 1: A New Spotlight on Inflammatory Diseases.Frontiers in immunology · 2021Review
- Integrated single cell analysis of blood and cerebrospinal fluid leukocytes in multiple sclerosis.Nature communications · 2020Article
- Brain and Hepatic Mt mRNA Is Reduced in Response to Mild Energy Restriction and n-3 Polyunsaturated Fatty Acid Deficiency in Juvenile Rats.Nutrients · 2017Article
- Metallothionein, Copper and Alpha-Synuclein in Alpha-Synucleinopathies.Frontiers in neuroscience · 2017Review
- Evaluation of enzyme dose and dose-frequency in ameliorating substrate accumulation in MPS IIIA Huntaway dog brain.Journal of inherited metabolic disease · 2015Article
- Lysosomal storage disease: gene therapy on both sides of the blood-brain barrier.Molecular genetics and metabolism · 2015Review
- Deregulation of subcellular biometal homeostasis through loss of the metal transporter, Zip7, in a childhood neurodegenerative disorder.Acta neuropathologica communications · 2014Article
- Systems-based analyses of brain regions functionally impacted in Parkinson's disease reveals underlying causal mechanisms.PloS one · 2014Article
Corrections and comments
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Authors and funding
13 authors at 5 institutions in 2 countries.
Funding
Abstract
objectiveTo facilitate development of novel disease-modifying therapies for lysosomal storage disorder (LSDs) characterized by nervous system involvement such as metachromatic leukodystrophy (MLD), molecular markers for monitoring disease progression and therapeutic response are needed. To this end, we sought to identify blood transcripts associated with the progression of MLD.
methodsGenome-wide expression analysis was performed in primary T lymphocytes of 24 patients with MLD compared to 24 age- and sex-matched healthy controls. Genes associated with MLD were identified, confirmed on a quantitative polymerase chain reaction platform, and replicated in an independent patient cohort. mRNA and protein expression of the prioritized gene family of metallothioneins was evaluated in postmortem patient brains and in mouse models representing 6 other LSDs. Metallothionein expression during disease progression and in response to specific treatment was evaluated in 1 of the tested LSD mouse models. Finally, a set of in vitro studies was planned to dissect the biological functions exerted by this class of molecules.
resultsMetallothionein genes were significantly overexpressed in T lymphocytes and brain of patients with MLD and generally marked nervous tissue damage in the LSDs here evaluated. Overexpression of metallothioneins correlated with measures of disease progression in mice and patients, whereas their levels decreased in mice upon therapeutic treatment. In vitro studies indicated that metallothionein expression is regulated in response to oxidative stress and inflammation, which are biochemical hallmarks of lysosomal storage diseases.
interpretationMetallothioneins are potential markers of neurologic disease processes and treatment response in LSDs.
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