Fsp1 Protein Ferroptosis Suppressor Protein 1 is an important component in the neurobiology of neurodegenerative diseases. This page provides detailed information about its structure, function, and role in disease processes.
Fsp1 Protein Ferroptosis Suppressor Protein 1 is an important component in the neurobiology of neurodegenerative diseases. This page provides detailed information about its structure, function, and role in disease processes.
<div class="infobox infobox-protein"> [@nadhdependent]
<table> [@fspa]
<tr><th colspan="2" style="background:#f0f0f0;">FSP1 Protein (Ferroptosis Suppressor Protein 1)</th></tr> [@coenzyme]
<tr><td><b>Protein Name</b></td><td>Ferroptosis Suppressor Protein 1 (FSP1)</td></tr> [^5]
<tr><td><b>Gene</b></td><td>[FSP1 (AIFM2)](/genes/fsp1)</td></tr>
<tr><td><b>UniProt ID</b></td><td>[Q9NSW9](https://www.uniprot.org/uniprot/Q9NSW9)</td></tr>
<tr><td><b>Alternative Names</b></td><td>AIFM2, Apoptosis-Inducing Factor Mitochondrial 2</td></tr>
<tr><td><b>Molecular Weight</b></td><td>~35 kDa</td></tr>
<tr><td><b>Subcellular Localization</b></td><td>Mitochondria, plasma membrane, peroxisomes</td></tr>
<tr><td><b>Protein Family</b></td><td>AIF family, NADH:quinone oxidoreductases</td></tr>
<tr><td><b>Structure</b></td><td>FAD/NADH binding domains</td></tr>
<tr>
<td class="label">Associated Diseases</td>
<td><a href="/wiki/als" style="color:#ef9a9a">Als</a>, <a href="/wiki/alzheimer" style="color:#ef9a9a">Alzheimer</a>, <a href="/wiki/cancer" style="color:#ef9a9a">Cancer</a>, <a href="/wiki/chronic-kidney-disease" style="color:#ef9a9a">Chronic Kidney Disease</a>, <a href="/wiki/fibrosis" style="color:#ef9a9a">Fibrosis</a></td>
</tr>
<tr>
<td class="label">KG Connections</td>
<td><a href="/atlas" style="color:#4fc3f7">129 edges</a></td>
</tr>
</table>
</div>
FSP1 ([Ferroptosis](/entities/ferroptosis) Suppressor Protein 1) is a NADH-dependent coenzyme Q reductase that suppresses ferroptosis through a GPX4-independent mechanism. Originally named AIFM2 ([Apoptosis](/entities/apoptosis) Factor Mitochondrial 2), it was renamed FSP1 due to its primary role in ferroptosis suppression. The protein catalyzes the reduction of CoQ10 to ubiquinol, which acts as a potent lipophilic antioxidant in membranes.
FSP1 contains key structural features:
The enzyme operates through a ping-pong mechanism:
FSP1 catalyzes:
Ubiquinol (CoQ10H2) generated by FSP1:
FSP1 provides GPX4-independent ferroptosis protection:
| Compound | Mechanism | Stage | Notes |
|----------|-----------|-------|-------|
| iFSP1 | Direct inhibition | Research | First selective FSP1 inhibitor |
| Screening compounds | Various | Discovery | Ongoing efforts |
| Strategy | Approach | Status |
|----------|----------|--------|
| NAD+ precursors | Increase NADH | Research |
| FSP1 expression | Gene therapy | Preclinical |
| CoQ10 supplementation | Substrate availability | Clinical trials |
| Partner | Interaction Type | Function |
|---------|-----------------|----------|
| CoQ10 | Substrate | Electron acceptor |
| NADH | Substrate | Electron donor |
| FAD | Cofactor | Electron transfer |
| Mitochondrial complex I | Metabolic link | Electron transport |
The study of Fsp1 Protein Ferroptosis Suppressor Protein 1 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.