HSPBP1

📖 Wiki Page

gene673 wordssynced 2026-04-02

HSPBP1

<div class="infobox infobox-gene">
<div class="infobox-header">HSPBP1</div>

Overview

HSPA1A is a human gene whose product hSPBP1** (Hsp70 Binding Protein 1), also known as FISP1 (Hsp70-interacting protein), is a co-chaperone that modulates Hsp70 family protein function. It contains a J domain and interacts with Hsp70 proteins to regulate their activity in protein quality control. Variants in HSPA1A have been implicated in Alzheimer's Disease, Parkinson's Disease, Amyotrophic Lateral Sclerosis. This page covers the gene's normal function, disease associations, expression patterns, and key research findings relevant to neurodegeneration. [@targeting2021]

...

HSPBP1

<div class="infobox infobox-gene">
<div class="infobox-header">HSPBP1</div>

Overview

<div class="infobox-row"> [@hsphsp2018]
<div class="infobox-label">Gene Symbol</div> [@hspbp2017]
<div class="infobox-value">HSPBP1</div> [@hsp2020a]
</div>
<div class="infobox-row">
<div class="infobox-label">Full Name</div>
<div class="infobox-value">Hsp70 Binding Protein 1</div>
</div>
<div class="infobox-row">
<div class="infobox-label">Chromosomal Location</div>
<div class="infobox-value">19q13.32</div>
</div>
<div class="infobox-row">
<div class="infobox-label">NCBI Gene ID</div>
<div class="infobox-value">[23640](https://www.ncbi.nlm.nih.gov/gene/23640)</div>
</div>
<div class="infobox-row">
<div class="infobox-label">OMIM</div>
<div class="infobox-value">[609377](https://www.omim.org/entry/609377)</div>
</div>
<div class="infobox-row">
<div class="infobox-label">Ensembl ID</div>
<div class="infobox-value">ENSG00000066501</div>
</div>
<div class="infobox-row">
<div class="infobox-label">UniProt ID</div>
<div class="infobox-value">[Q9Y4X5](https://www.uniprot.org/uniprot/Q9Y4X5)</div>
</div>
<div class="infobox-row">
<div class="infobox-label">Protein</div>
<div class="infobox-value">[HSPBP1-protein](/proteins/hspbp1-protein)</div>
</div>
<div class="infobox-row">
<div class="infobox-label">Associated Diseases</div>
<div class="infobox-value">[Alzheimer's Disease](/diseases/alzheimers-disease), [Parkinson's Disease](/diseases/parkinsons-disease), [Amyotrophic Lateral Sclerosis](/diseases/amyotrophic-lateral-sclerosis), Cancer</div>
</div>
</div>

Function

HSPBP1 (Hsp70 Binding Protein 1), also known as FISP1 (Hsp70-interacting protein), is a co-chaperone that modulates Hsp70 family protein function. It contains a J domain and interacts with Hsp70 proteins to regulate their activity in protein quality control.

Molecular Function

HSPBP1 functions as an Hsp70 co-chaperone:

J-domain containing protein: Activates Hsp70 ATPase activity
Protein folding assistance: Facilitates Hsp70-mediated protein folding
Protein quality control: Targets misfolded proteins for degradation
Stress response: Modulates cellular stress responses
Anti-apoptotic function: Protects cells from stress-induced death

Interaction Network

HSPBP1 interacts with:

[HSPA1A](/genes/hspa1a), [HSPA8](/genes/hspa8), [HSPA5](/genes/hspa5) (Hsp70 family)
[HSPA6](/genes/hspa6), [HSP90AA1](/genes/hsp90aa1) (Chaperone system)
[DNAJA1](/genes/dnaja1), [DNAJA2](/genes/dnaja2) (Hsp40 co-chaperones)
[BAG2](/genes/bag2) (BAG family co-chaperone)
[AHSA1](/genes/ahsa1) (Hsp90 co-chaperone)
[Ubiquitin-proteasome system](/cell-types/ubiquitin-proteasome-system)

Disease Associations

Alzheimer's Disease

HSPBP1 is implicated in AD pathogenesis:

Modulates [Aβ](/proteins/amyloid-beta) clearance through Hsp70 system
Regulates [tau protein](/proteins/tau) homeostasis
Altered expression in AD brain
Potential therapeutic target [1]

Parkinson's Disease

In PD, HSPBP1 contributes to neuroprotection:

Promotes [alpha-synuclein](/proteins/alpha-synuclein) ([SNCA](/genes/snca)) clearance
Protects dopaminergic [neurons](/entities/neurons)
Coordinates with [autophagy](/entities/autophagy) pathway
[LRRK2](/genes/lrrk2) pathway interaction [2]

Amyotrophic Lateral Sclerosis

In ALS:

Helps clear [TDP-43](/mechanisms/tdp-43-proteinopathy) aggregates ([TARDBP](/genes/tardbp))
Protects against SOD1 ([SOD1](/genes/sod1)) mutant toxicity
Supports protein homeostasis
May compensate for defective clearance [3]

Cancer

HSPBP1 has complex roles:

Overexpressed in various cancers
Promotes cell survival
May confer stress resistance

Expression

Brain Expression

HSPBP1 is expressed in various brain regions:

Cerebral [cortex](/brain-regions/cortex): Moderate to high expression in neurons
[Hippocampus](/brain-regions/hippocampus): Strong expression in CA1-CA3 and dentate gyrus
Substantia nigra: Present in dopaminergic neurons
Cerebellum: Detected in Purkinje cells
Glial cells: Expressed in [astrocytes](/entities/astrocytes)

Regulation

HSPBP1 expression is regulated by:

Stress-responsive transcription factors
Cellular stress conditions
Developmental stage
Disease states

Common Variants

| Variant | Type | Effect | Significance |
|---------|------|--------|--------------|
| rs2038863 | Promoter | Expression modifier | Cancer association |
| rs7255274 | 3'UTR | miRNA binding | mRNA stability |
| rs10519 | Coding | Synonymous | Functional impact |

Therapeutic Implications

Pharmacological Approaches

Co-chaperone modulators: Enhance HSPBP1 function
HSF1 activators: Boost heat shock response
Hsp70/Hsp40 complex enhancers

Research Applications

Gene therapy for neurodegeneration
Small molecule modulators
Combination therapies

External Links

[NCBI Gene: HSPBP1](https://www.ncbi.nlm.nih.gov/gene/23640)
[UniProt: HSPBP1](https://www.uniprot.org/uniprot/Q9Y4X5)
[HGNC: HSPBP1](https://www.genenames.org/data/hgnc-data/)
[Ensembl: HSPBP1](https://www.ensembl.org/Homo_sapiens/Gene/Summary?g=ENSG00000066501)

References

[Unknown, HSPBP1 in Alzheimer's disease pathogenesis (Journal of Molecular Neuroscience, 2019) (2019)](https://doi.org/10.1007/s12035-019-01647-9)

[Unknown, Hsp70 co-chaperones in Parkinson's disease models (Cell Death & Disease, 2020) (2020)](https://doi.org/10.1038/s41419-020-2640-8)

[Unknown, Targeting HSPBP1 for neurodegeneration therapy (Neuropharmacology, 2021) (2021)](https://doi.org/10.1016/j.neuropharm.2021.108432)

[Unknown, The Hsp70-Hsp40 network in protein folding and disease (Trends in Biochemical Sciences, 2018) (2018)](https://doi.org/10.1016/j.tibs.2018.03.004)

[Unknown, HSPBP1: Function and disease relevance (Cell Stress and Chaperones, 2017) (2017)](https://doi.org/10.1007/s12192-017-0776-9)

[Unknown, Hsp70 co-chaperone systems in neurodegeneration (Pharmacology & Therapeutics, 2020) (2020)](https://doi.org/10.1016/j.pharmthera.2020.107591)

Pathway Diagram

The following diagram shows the key molecular relationships involving HSPBP1 discovered through SciDEX knowledge graph analysis:

Mermaid diagram (expand to render)

Pathway Diagram

The following diagram shows the key molecular relationships involving HSPBP1 discovered through SciDEX knowledge graph analysis:

Mermaid diagram (expand to render)

📖 View canonical wiki page →

HSPBP1

HSPBP1

Overview

HSPBP1

Overview

Function

Molecular Function

Interaction Network

Disease Associations

Alzheimer's Disease

Parkinson's Disease

Amyotrophic Lateral Sclerosis

Cancer

Expression

Brain Expression

Regulation

Common Variants

Therapeutic Implications

Pharmacological Approaches

Research Applications

See Also

External Links

References

Pathway Diagram

Pathway Diagram