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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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HSPBP1
HSPA (Hsp70) binding protein 1
Chromosome 19 · 19q13.42
NCBI Gene: 23640Ensembl: ENSG00000133265.12HGNC: HGNC:24989UniProt: Q9NZL4
131PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Hub Gene
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
positive regulation of proteasomal ubiquitin-dependent protein catabolic processpositive regulation of protein ubiquitinationGO:0005615protein bindingneurodegenerative diseasehypothyroidismazoospermiapartial chromosome Y deletion
✦AI Summary

HSPBP1 (HSPA binding protein 1) is a cochaperone that regulates Hsp70 chaperone activity through nucleotide exchange factor (NEF) activity 1. Primary function: HSPBP1 inhibits Hsp70-mediated protein folding by preventing ATP binding to the chaperone's nucleotide-binding domain 1, and interferes with STUB1-mediated ubiquitination, thereby protecting misfolded proteins like immature CFTR from degradation. Beyond canonical chaperone regulation, HSPBP1 functions as a stress granule component that promotes their assembly during cellular stress through interactions with G3BP1, HuR, and TIA-1/TIAR proteins 2. HSPBP1 also inhibits formation of cytotoxic Tag7-Hsp70 complexes in serum, potentially protecting normal cells from immune attack 3. Differentially from related NEF BAG-1M, HSPBP1 inhibits steroid receptor activity across glucocorticoid, mineralocorticoid, and androgen receptors 4. Disease relevance: During HIV-1 infection, the virus downregulates HSPBP1 expression through its Tat transactivator, enhancing viral replication 5. Elevated serum HSPBP1 and anti-HSPBP1 antibodies correlate with HIV disease progression and CD4 depletion 6. Clinical significance: HSPBP1 quantification may serve as an adjunctive HIV progression marker 6, complementing established clinical parameters.

Sources cited
1
HSPBP1 functions as a nucleotide exchange factor for Hsp70, facilitating conversion from ADP-bound to ATP-bound state
PMID: 25487014
2
HSPBP1 is an integral stress granule component that regulates SG formation through interactions with G3BP1, HuR, TIA-1/TIAR, and polyA-RNA
PMID: 32235396
3
Extracellular HSPBP1 inhibits formation of cytotoxic Tag7-Hsp70 complexes in serum and conditioned medium
PMID: 22037021
4
HSPBP1 inhibits steroid receptor (GR, MR, AR) activity and reduces Hsp70 binding to receptor ligand-binding domains, differentially from BAG-1M
PMID: 24454860
5
HIV-1 Tat protein downregulates HSPBP1 promoter activity by blocking Sp1 recruitment, suppressing HSPBP1 expression during infection
PMID: 36581045
6
Serum HSPBP1 and anti-HSPBP1 levels correlate negatively with CD4 counts and CD4:CD8 ratio in HIV-infected individuals, associating with disease progression
PMID: 30786116
Disease Associationsⓘ20
neurodegenerative diseaseOpen Targets
0.37Weak
hypothyroidismOpen Targets
0.11Weak
azoospermiaOpen Targets
0.09Suggestive
partial chromosome Y deletionOpen Targets
0.08Suggestive
breast cancerOpen Targets
0.08Suggestive
spinocerebellar ataxia type 32Open Targets
0.07Suggestive
spermatogenic failure 50Open Targets
0.06Suggestive
spermatogenic failure 57Open Targets
0.06Suggestive
spermatogenic failure 25Open Targets
0.06Suggestive
isochromosomy YpOpen Targets
0.06Suggestive
spermatogenic failure 71Open Targets
0.06Suggestive
spermatogenic failure, X-linked, 2Open Targets
0.06Suggestive
congenital bilateral absence of vas deferensOpen Targets
0.06Suggestive
spermatogenic failure 63Open Targets
0.06Suggestive
isochromosomy YqOpen Targets
0.06Suggestive
spermatogenic failure 61Open Targets
0.06Suggestive
spermatogenic failure 62Open Targets
0.06Suggestive
spermatogenic failure 88Open Targets
0.06Suggestive
spermatogenic failure 59Open Targets
0.06Suggestive
spermatogenic failure 73Open Targets
0.06Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
HSP90AA1Protein interaction100%HSP90AB1Protein interaction100%FAUProtein interaction100%RPS3AProtein interaction100%RPS3Protein interaction100%RPS11Protein interaction100%
Tissue Expression6 tissues
Liver
100%
Ovary
94%
Brain
82%
Bone Marrow
66%
Lung
61%
Heart
61%
Gene Interaction Network
Click a node to explore
HSPBP1HSP90AA1HSP90AB1FAURPS3ARPS3RPS11
PROTEIN STRUCTURE
Preparing viewer…
PDB1XQR · 2.10 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
1.17LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.86 [0.65–1.17]
RankingsWhere HSPBP1 stands among ~20K protein-coding genes
  • #3,564of 20,598
    Most Researched131 · top quartile
  • #12,208of 17,882
    Most Constrained (LOEUF)1.17
Genes detectedHSPBP1
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Identification of 5' upstream sequence involved in HSPBP1 gene transcription and its downregulation during HIV-1 infection.
PMID: 36581045
Virus Res · 2023
1.00
2
HspBP1 and anti-HspBP1 levels in the serum of HIV-infected individuals are associated to the disease progression.
PMID: 30786116
J Appl Microbiol · 2019
0.90
3
Extracellular HspBP1 inhibits formation of a cytotoxic Tag7-Hsp70 complex in vitro and in human serum.
PMID: 22037021
Biochimie · 2012
0.80
4
Effect of exercise on the expression of HSPBP1, PGLYRP1, and HSPA1A genes in human leukocytes.
PMID: 23113305
Bull Exp Biol Med · 2012
0.70
5
Human serum contains detectable levels of the Hsp70 cochaperone HspBP1 and antibodies bound to HspBP1.
PMID: 16827227
J Immunoassay Immunochem · 2006
0.60