ReviewJournal of inherited metabolic disease2015
Defective lipid metabolism in neurodegeneration with brain iron accumulation (NBIA) syndromes: not only a matter of iron.
Review in Journal of inherited metabolic disease, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 21 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Who cites it
21 citing papers in PubMed, 49 citations in OpenAlex.
- Article
- Serum metabolomics indicates ferroptosis in patients with pantothenate kinase associated neurodegeneration.Scientific reports · 2025Article
- Impaired mitochondrial integrity and compromised energy production underscore the mechanism underlying CoASY protein-associated neurodegeneration.Cellular and molecular life sciences : CMLS · 2025Article
- Genetic Targets and Applications of Iron Chelators for Neurodegeneration with Brain Iron Accumulation.ACS bio & med chem Au · 2024Review
- Apolipoprotein E potently inhibits ferroptosis by blocking ferritinophagy.Molecular psychiatry · 2024Article
- Dyslipidemia and hypercalciuria in a patient with pantothenate kinase 2 deficiency: A novel variant and case report.SAGE open medical case reports · 2024Article
- Remodeling of astrocyte secretome in amyotrophic lateral sclerosis: uncovering novel targets to combat astrocyte-mediated toxicity.Translational neurodegeneration · 2022Review
- Pesticides Exposure-Induced Changes in Brain Metabolome: Implications in the Pathogenesis of Neurodegenerative Disorders.Neurotoxicity research · 2022Review
- PKAN hiPS-Derived Astrocytes Show Impairment of Endosomal Trafficking: A Potential Mechanism Underlying Iron Accumulation.Frontiers in cellular neuroscience · 2022Article
- New Insights of Phospholipase A2 Associated Neurodegeneration Phenotype Based on the Long-Term Follow-Up of a Large Hungarian Family.Frontiers in genetics · 2021Article
- Cerebral imaging in paediatric mitochondrial disorders.The neuroradiology journal · 2018Review
- Transcranial Sonography in Mitochondrial Membrane Protein-Associated Neurodegeneration.Clinical neuroradiology · 2018Article
- Synthesis and neuroprotective effects of the complex nanoparticles of iron and sapogenin isolated from the defatted seeds of Camellia oleifera.Pharmaceutical biology · 2017Article
- Down-regulation of coasy, the gene associated with NBIA-VI, reduces Bmp signaling, perturbs dorso-ventral patterning and alters neuronal development in zebrafish.Scientific reports · 2016Article
- Coenzyme A corrects pathological defects in human neurons of PANK2-associated neurodegeneration.EMBO molecular medicine · 2016Article
- Clinical and imaging characteristics of late onset mitochondrial membrane protein-associated neurodegeneration (MPAN).Neurocase · 2016Article
- Contribution of metals to brain MR signal intensity: review articles.Japanese journal of radiology · 2016Review
- Autophagy in Neurodegenerative Diseases and Metal Neurotoxicity.Neurochemical research · 2016Review
- Modeling human Coenzyme A synthase mutation in yeast reveals altered mitochondrial function, lipid content and iron metabolism.Microbial cell (Graz, Austria) · 2015Article
- Article
Corrections and comments
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Authors and funding
3 authors at 1 institution in 1 country.
Funding
Abstract
Neurodegeneration with brain iron accumulation (NBIA) is a group of devastating and life threatening rare diseases. Adult and early-onset NBIA syndromes are inherited as X-chromosomal, autosomal dominant or recessive traits and several genes have been identified as responsible for these disorders. Among the identified disease genes, only two code for proteins directly involved in iron metabolism while the remaining NBIA genes encode proteins with a wide variety of functions ranging from fatty acid metabolism and autophagy to still unknown activities. It is becoming increasingly evident that many neurodegenerative diseases are associated with metabolic dysfunction that often involves altered lipid metabolism. This is not surprising since neurons have a peculiar and heterogeneous lipid composition critical for the development and correct functioning of the nervous system. This review will focus on specific NBIA forms, namely PKAN, CoPAN, PLAN, FAHN and MPAN, which display an interesting link between neurodegeneration and alteration of phospholipids and sphingolipids metabolism, mitochondrial morphology and membrane remodelling.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.