Fundamental Neurochemistry Review: Copper availability as a potential therapeutic target in progressive supranuclear palsy: Insight from other neurodegenerative diseases.
- Publisher:
- WILEY
- Publication Type:
- Journal Article
- Citation:
- J Neurochem, 2023, 167, (3), pp. 337-346
- Issue Date:
- 2023-11
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Full metadata record
Field | Value | Language |
---|---|---|
dc.contributor.author | Billings, JL | |
dc.contributor.author | Hilton, JBW | |
dc.contributor.author | Liddell, JR | |
dc.contributor.author | Hare, DJ | |
dc.contributor.author | Crouch, PJ | |
dc.date.accessioned | 2024-03-25T23:38:41Z | |
dc.date.available | 2023-09-08 | |
dc.date.available | 2024-03-25T23:38:41Z | |
dc.date.issued | 2023-11 | |
dc.identifier.citation | J Neurochem, 2023, 167, (3), pp. 337-346 | |
dc.identifier.issn | 0022-3042 | |
dc.identifier.issn | 1471-4159 | |
dc.identifier.uri | http://hdl.handle.net/10453/177144 | |
dc.description.abstract | Since the first description of Parkinson's disease (PD) over two centuries ago, the recognition of rare types of atypical parkinsonism has introduced a spectrum of related PD-like diseases. Among these is progressive supranuclear palsy (PSP), a neurodegenerative condition that clinically differentiates through the presence of additional symptoms uncommon in PD. As with PD, the initial symptoms of PSP generally present in the sixth decade of life when the underpinning neurodegeneration is already significantly advanced. The causal trigger of neuronal cell loss in PSP is unknown and treatment options are consequently limited. However, converging lines of evidence from the distinct neurodegenerative conditions of PD and amyotrophic lateral sclerosis (ALS) are beginning to provide insights into potential commonalities in PSP pathology and opportunity for novel therapeutic intervention. These include accumulation of the high abundance cuproenzyme superoxide dismutase 1 (SOD1) in an aberrant copper-deficient state, associated evidence for altered availability of the essential micronutrient copper, and evidence for neuroprotection using compounds that can deliver available copper to the central nervous system. Herein, we discuss the existing evidence for SOD1 pathology and copper imbalance in PSP and speculate that treatments able to provide neuroprotection through manipulation of copper availability could be applicable to the treatment of PSP. | |
dc.format | Print-Electronic | |
dc.language | eng | |
dc.publisher | WILEY | |
dc.relation.ispartof | J Neurochem | |
dc.relation.isbasedon | 10.1111/jnc.15978 | |
dc.rights | info:eu-repo/semantics/openAccess | |
dc.subject | 0601 Biochemistry and Cell Biology, 1109 Neurosciences | |
dc.subject.classification | Neurology & Neurosurgery | |
dc.subject.classification | 3101 Biochemistry and cell biology | |
dc.subject.classification | 3209 Neurosciences | |
dc.subject.mesh | Humans | |
dc.subject.mesh | Supranuclear Palsy, Progressive | |
dc.subject.mesh | Copper | |
dc.subject.mesh | Neurodegenerative Diseases | |
dc.subject.mesh | Superoxide Dismutase-1 | |
dc.subject.mesh | Neurochemistry | |
dc.subject.mesh | Parkinson Disease | |
dc.subject.mesh | Humans | |
dc.subject.mesh | Parkinson Disease | |
dc.subject.mesh | Supranuclear Palsy, Progressive | |
dc.subject.mesh | Neurodegenerative Diseases | |
dc.subject.mesh | Copper | |
dc.subject.mesh | Neurochemistry | |
dc.subject.mesh | Superoxide Dismutase-1 | |
dc.subject.mesh | Humans | |
dc.subject.mesh | Supranuclear Palsy, Progressive | |
dc.subject.mesh | Copper | |
dc.subject.mesh | Neurodegenerative Diseases | |
dc.subject.mesh | Superoxide Dismutase-1 | |
dc.subject.mesh | Neurochemistry | |
dc.subject.mesh | Parkinson Disease | |
dc.title | Fundamental Neurochemistry Review: Copper availability as a potential therapeutic target in progressive supranuclear palsy: Insight from other neurodegenerative diseases. | |
dc.type | Journal Article | |
utslib.citation.volume | 167 | |
utslib.location.activity | England | |
utslib.for | 0601 Biochemistry and Cell Biology | |
utslib.for | 1109 Neurosciences | |
pubs.organisational-group | University of Technology Sydney | |
pubs.organisational-group | University of Technology Sydney/Faculty of Science | |
pubs.organisational-group | University of Technology Sydney/Faculty of Science/School of Mathematical and Physical Sciences | |
utslib.copyright.status | open_access | * |
dc.date.updated | 2024-03-25T23:38:39Z | |
pubs.issue | 3 | |
pubs.publication-status | Published | |
pubs.volume | 167 | |
utslib.citation.issue | 3 |
Abstract:
Since the first description of Parkinson's disease (PD) over two centuries ago, the recognition of rare types of atypical parkinsonism has introduced a spectrum of related PD-like diseases. Among these is progressive supranuclear palsy (PSP), a neurodegenerative condition that clinically differentiates through the presence of additional symptoms uncommon in PD. As with PD, the initial symptoms of PSP generally present in the sixth decade of life when the underpinning neurodegeneration is already significantly advanced. The causal trigger of neuronal cell loss in PSP is unknown and treatment options are consequently limited. However, converging lines of evidence from the distinct neurodegenerative conditions of PD and amyotrophic lateral sclerosis (ALS) are beginning to provide insights into potential commonalities in PSP pathology and opportunity for novel therapeutic intervention. These include accumulation of the high abundance cuproenzyme superoxide dismutase 1 (SOD1) in an aberrant copper-deficient state, associated evidence for altered availability of the essential micronutrient copper, and evidence for neuroprotection using compounds that can deliver available copper to the central nervous system. Herein, we discuss the existing evidence for SOD1 pathology and copper imbalance in PSP and speculate that treatments able to provide neuroprotection through manipulation of copper availability could be applicable to the treatment of PSP.
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