Opa1 Protein is an important component in the neurobiology of neurodegenerative diseases. This page provides detailed information about its structure, function, and role in disease processes.
| OPA1 Protein |
|---|
| Protein Name | Optic Atrophy 1 |
| Gene | OPA1 |
| UniProt ID | O60313 |
| PDB IDs | 5W5V, 6JTG |
| Molecular Weight | 89-100 kDa (isoforms) |
| Subcellular Localization | Inner mitochondrial membrane |
| Protein Family | Dynamin-related GTPases |
OPA1 PROTEIN is a gene/protein encoding a key neuronal protein involved in synaptic function, signal transduction, and cellular homeostasis. Dysfunction of OPA1 PROTEIN is associated with neurodegenerative diseases including Alzheimer's disease, Parkinson's disease, and related disorders.
OPA1 is a dynamin-related GTPase with:
- N-terminal GTPase domain: Catalytic GTP hydrolysis
- Middle domain: Dimerization
- GTPase effector domain (GED): Regulates assembly
- Transmembrane anchor: Inner membrane localization
- Isoforms: Multiple isoforms from alternative splicing
- Inner membrane fusion: Mediates mitochondrial inner membrane fusion
- Cristae maintenance: Preserves cristae structure and tightness
- mtDNA nucleoids: Essential for mitochondrial DNA organization
- Apoptosis control: Inhibits cytochrome c release
- Metabolic regulation: Maintains oxidative phosphorylation
- Most common inherited optic neuropathy
- Primary cause is OPA1 mutations (60-70%)
- Selective retinal ganglion cell degeneration
- Mitochondrial fragmentation in PD
- Interaction with PINK1/Parkin pathway
- Dopaminergic neuron vulnerability
- Additional neurological manifestations
- Hearing loss, peripheral neuropathy
| Approach |
Status |
Description |
| Gene therapy |
Research |
AAV-OPA1 delivery |
| Small molecule modulators |
Research |
Enhancing OPA1 function |
| Mitochondrial protectants |
Research |
General mitochondrial protection |
- 10853840: OPA1 mutations cause DOA. Nat Genet, 2000.
- 18581056: OPA1 in mitochondrial dynamics. Nat Rev Neurosci, 2008.
- 26791242: OPA1 neuroprotection. J Neurochem, 2016.
The study of Opa1 Protein has evolved significantly over the past decades. Research in this area has revealed important insights into the underlying mechanisms of neurodegeneration and continues to drive therapeutic development.
Historical context and key discoveries in this field have shaped our current understanding and will continue to guide future research directions.
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- Herkenne S et al.. "Developmental and Tumor Angiogenesis Requires the Mitochondria-Shaping Protein Opa1." Cell metabolism (2020). DOI: 10.1016/j.cmet.2020.04.007 PubMed: 32315597
- Nasoni MG et al.. "Melatonin reshapes the mitochondrial network and promotes intercellular mitochondrial transfer via tunneling nanotubes after ischemic-like injury in hippocampal HT22 cells." Journal of pineal research (2021). DOI: 10.1111/jpi.12747 PubMed: 34085316
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- Chang X et al.. "ROS-Drp1-mediated mitochondria fission contributes to hippocampal HT22 cell apoptosis induced by silver nanoparticles." Redox biology (2023). DOI: 10.1016/j.redox.2023.102739 PubMed: 37187014
- Salminen A et al.. "Impaired autophagy and APP processing in Alzheimer's disease: The potential role of Beclin 1 interactome." Progress in neurobiology (2013). DOI: 10.1016/j.pneurobio.2013.06.002 PubMed: 23827971