ELOVL7 Protein

ELOVL7 Protein - Elongation of Very Long Chain Fatty Acids 7

Introduction

Elovl7 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.

<div class="infobox infobox-protein">
<table><tr><th colspan="2" style="background:#f0f0f0; text-align:center;">ELOVL7</th></tr>
<tr><td><b>Gene Symbol</b></td><td>[ELOVL7](/genes/elovl7)</td></tr>
<tr><td><b>UniProt ID</b></td><td>[Q9H8Q0](https://www.uniprot.org/uniprotkb/Q9H8Q0/entry)</td></tr>
<tr><td><b>PDB ID</b></td><td>5D51, 5D52</td></tr>
<tr><td><b>Molecular Weight</b></td><td>33.6 kDa</td></tr>
<tr><td><b>Subcellular Localization</b></td><td>Endoplasmic reticulum membrane</td></tr>
<tr><td><b>Protein Family</b></td><td>ELOVL (Elongation of Very Long Chain Fatty Acids) family</td></tr>
<tr>
<td class="label">KG Connections</td>
<td><a href="/atlas" style="color:#4fc3f7">1 edges</a></td>
</tr>
</table>
</div>

Overview

ELOVL7 (Elongation of Very Long Chain Fatty Acids 7) is an endoplasmic reticulum membrane enzyme that catalyzes the rate-limiting step of very long-chain fatty acid (VLCFA) synthesis[@jakobsson2006]. It is encoded by the [ELOVL7](/proteins/elovl7-protein) gene on chromosome 5p13.3 and plays critical roles in lipid metabolism, membrane biology, and myelin formation.

Normal Function

...

ELOVL7 Protein - Elongation of Very Long Chain Fatty Acids 7

Introduction

Elovl7 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.

<div class="infobox infobox-protein">
<table><tr><th colspan="2" style="background:#f0f0f0; text-align:center;">ELOVL7</th></tr>
<tr><td><b>Gene Symbol</b></td><td>[ELOVL7](/genes/elovl7)</td></tr>
<tr><td><b>UniProt ID</b></td><td>[Q9H8Q0](https://www.uniprot.org/uniprotkb/Q9H8Q0/entry)</td></tr>
<tr><td><b>PDB ID</b></td><td>5D51, 5D52</td></tr>
<tr><td><b>Molecular Weight</b></td><td>33.6 kDa</td></tr>
<tr><td><b>Subcellular Localization</b></td><td>Endoplasmic reticulum membrane</td></tr>
<tr><td><b>Protein Family</b></td><td>ELOVL (Elongation of Very Long Chain Fatty Acids) family</td></tr>
<tr>
<td class="label">KG Connections</td>
<td><a href="/atlas" style="color:#4fc3f7">1 edges</a></td>
</tr>
</table>
</div>

Overview

ELOVL7 (Elongation of Very Long Chain Fatty Acids 7) is an endoplasmic reticulum membrane enzyme that catalyzes the rate-limiting step of very long-chain fatty acid (VLCFA) synthesis[@jakobsson2006]. It is encoded by the [ELOVL7](/proteins/elovl7-protein) gene on chromosome 5p13.3 and plays critical roles in lipid metabolism, membrane biology, and myelin formation.

Normal Function

ELOVL7 catalyzes the first step of very long-chain fatty acid (VLCFA) elongation, converting C14-C16 fatty acids to C18-C26+ fatty acids through a four-step condensation reaction:

Condensation: Malonyl-CoA adds two carbons to the fatty acid chain

Reduction: NADPH reduces the β-keto group

Dehydration: Removal of water to form a trans-Δ2 double bond

Reduction: Another NADPH reduces the trans-Δ2 enoyl-CoA

Substrate Specificity

ELOVL7 has broad substrate specificity:

• Preferred substrates: C14-C16 acyl-CoAs
• Can elongate: C12 to C22+ fatty acids
• Products: C18 to C26+ VLCFAs

Role in Lipid Metabolism

VLCFAs synthesized by ELOVL7 are essential for:

• Phospholipids: Major component of neuronal membranes
• Sphingolipids: Myelin structure and function
• Cholesterol esters: Membrane fluidity
• Triacylglycerols: Energy storage

Structure

ELOVL7 contains several key structural features:

• N-terminal domain: Cytosolic facing, substrate binding
• Transmembrane domains: 6-7 passes through ER membrane
• Active site: HXXHH motif for catalysis
• C-terminal domain: ER retention signal

Crystal structures have revealed the catalytic mechanism and substrate access channel[@suna2020].

Expression Pattern

Brain Regional Distribution

ELOVL7 is expressed in:

• White matter: Highest in myelin-producing oligodendrocytes
• Cerebral [cortex](/brain-regions/cortex): [Neurons](/entities/neurons) and [astrocytes](/entities/astrocytes)
• [Hippocampus](/brain-regions/hippocampus): Particularly CA3 region
• Cerebellum: Purkinje cells and granule cells
• Peripheral nervous system: Schwann cells

Cellular Localization

• Endoplasmic reticulum: Site of VLCFA synthesis
• Myelin sheaths: Rich in VLCFA-containing lipids
• Lipid droplets: Storage form of VLCFAs

Disease Associations

Alzheimer's Disease

ELOVL7 in Alzheimer's disease[@international2015]:

• GWAS identifies ELOVL7 variants associated with AD risk
• Altered VLCFA metabolism in AD brain
• Role in [amyloid precursor protein](/entities/app-protein) (APP) processing
• Abnormalities in membrane lipid composition

Parkinson's Disease

• Lipid dysregulation in PD substantia nigra
• ELOVL7 variants associated with PD risk
• Role in [alpha-synuclein](/mechanisms/alpha-synuclein) membrane interactions
• Mitochondrial lipid metabolism impairment

Hereditary Spastic Paraplegia (HSP)

• VLCFA accumulation in some HSP subtypes
• ELOVL7 variants may modify disease severity
• Lipid metabolism defects in axonal degeneration

Other Neurological Conditions

• Multiple Sclerosis: Myelin lipid abnormalities
• Amyotrophic Lateral Sclerosis: Lipid metabolism alterations
• Bipolar Disorder: GWAS associations

Therapeutic Targeting

Small Molecule Modulators

| Agent | Target | Development Status | Indication |
|-------|--------|-------------------|------------|
| ELOVL7 inhibitors | Catalytic site | Preclinical | VLCFA reduction |
| Substrate analogs | Substrate binding | Discovery | Metabolic disorders |
| Gene therapy | ELOVL7 expression | Preclinical | Enzyme deficiency |

Therapeutic Strategies

VLCFA reduction: Lower elevated VLCFA levels

Membrane stabilization: Restore lipid composition

Myelin protection: Support oligodendrocyte function

Research Directions

Biomarkers

• ELOVL7 expression in CSF as lipid metabolism marker
• Plasma VLCFA levels
• Lipidomic profiles

Animal Models

• Elovl7 knockout mice: Viable with lipid abnormalities
• Transgenic mice: Overexpression studies
• Zebrafish models: Developmental studies

See Also

• ELOVL7 Gene
• [Lipid Metabolism Pathway](/mechanisms/lipid-metabolism-neurodegeneration)
• Myelin Pathway
• [Alzheimer's Disease](/diseases/alzheimers-disease)
• [Parkinson's Disease](/diseases/parkinsons-disease)
• [Hereditary Spastic Paraplegia](/diseases/hereditary-spastic-paraplegia)