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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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SH3BP5
SH3 domain binding protein 5
Chromosome 3 · 3p25.1
NCBI Gene: 9467Ensembl: ENSG00000131370.17HGNC: HGNC:10827UniProt: O60239
46PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
nuclear bodyprotein kinase inhibitor activityguanyl-nucleotide exchange factor activityprotein bindingovarian dysfunctionobesityneurodegenerative diseasetympanic membrane disease
✦AI Summary

SH3BP5 (SH3 domain binding protein 5) functions primarily as a guanine nucleotide exchange factor (GEF) with specificity for RAB11A and RAB25, facilitating GDP release and promoting GTP binding to regulate membrane trafficking 1. The protein exhibits a V-shaped structure comprising two coiled coils, with the coiled coil containing α1 and α4 helices being solely responsible for Rab11a binding and GEF activity 1. SH3BP5 localizes to Rab11-positive recycling endosomes and shows GEF activity for all Rab11 family members but not for Rab14 1. Additionally, SH3BP5 serves as a negative regulator of BTK kinase, inhibiting its auto- and transphosphorylation activity and playing a regulatory role in BTK-related cytoplasmic signaling in B-cells 2. The protein mediates acetaminophen hepatotoxicity through mitochondrial dysfunction and JNK phosphorylation 3. In cancer contexts, SH3BP5 expression is associated with poor prognosis in diffuse large B-cell lymphoma, particularly the ABC subtype, where it correlates with advanced-stage disease, elderly onset, and immunosuppressive microenvironment 45. The protein appears to promote metabolic reprogramming toward oxidative phosphorylation and may contribute to therapy resistance 4.

Sources cited
1
SH3BP5 functions as a GEF for RAB11A/RAB25, exhibits V-shaped coiled coil structure, and localizes to recycling endosomes
PMID: 30872413
2
SH3BP5 serves as a negative regulator of BTK kinase activity in B-cell signaling
PMID: 19161832
3
SH3BP5 mediates acetaminophen hepatotoxicity through mitochondrial dysfunction and JNK signaling
PMID: 31029706
4
SH3BP5 is associated with poor prognosis in DLBCL ABC subtype and promotes immunosuppressive microenvironment
PMID: 40993727
5
SH3BP5 expression correlates with advanced-stage disease and elderly onset in DLBCL patients
PMID: 27184832
Disease Associationsⓘ20
ovarian dysfunctionOpen Targets
0.32Weak
obesityOpen Targets
0.32Weak
neurodegenerative diseaseOpen Targets
0.25Weak
tympanic membrane diseaseOpen Targets
0.23Weak
arthritisOpen Targets
0.22Weak
hypertensionOpen Targets
0.14Weak
neoplasmOpen Targets
0.10Suggestive
diffuse large B-cell lymphomaOpen Targets
0.09Suggestive
Melnick-Needles syndromeOpen Targets
0.08Suggestive
breast cancerOpen Targets
0.08Suggestive
pachyonychia congenitaOpen Targets
0.08Suggestive
non-small cell lung carcinomaOpen Targets
0.08Suggestive
acute myeloid leukemiaOpen Targets
0.08Suggestive
infectionOpen Targets
0.04Suggestive
lung adenocarcinomaOpen Targets
0.04Suggestive
melanomaOpen Targets
0.04Suggestive
Alzheimer diseaseOpen Targets
0.03Suggestive
chronic obstructive pulmonary diseaseOpen Targets
0.03Suggestive
Huntington diseaseOpen Targets
0.02Suggestive
non-alcoholic steatohepatitisOpen Targets
0.02Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
MYZAPShared pathway100%LRRC69Shared pathway100%DCDC2CShared pathway100%LRRC18Shared pathway100%ASB18Shared pathway100%LRRC30Shared pathway100%
Tissue Expression6 tissues
Brain
100%
Ovary
86%
Lung
61%
Bone Marrow
40%
Heart
22%
Liver
8%
Gene Interaction Network
Click a node to explore
SH3BP5MYZAPLRRC69DCDC2CLRRC18ASB18LRRC30
PROTEIN STRUCTURE
Preparing viewer…
PDB4H3B · 2.08 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.85LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.61 [0.44–0.85]
RankingsWhere SH3BP5 stands among ~20K protein-coding genes
  • #9,379of 20,598
    Most Researched46
  • #7,377of 17,882
    Most Constrained (LOEUF)0.85
Genes detectedSH3BP5
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Endoplasmic Reticulum Stress-Induced Upregulation of STARD1 Promotes Acetaminophen-Induced Acute Liver Failure.
PMID: 31029706
Gastroenterology · 2019
1.00
2
LncRNA SH3BP5-AS1 promotes hepatocellular carcinoma progression by sponging miR-6838-5p and activation of PTPN4.
PMID: 38761175
Aging (Albany NY) · 2024
0.90
3
SH3BP5-driven metabolic-immune crosstalk in DLBCL: a prognostic biomarker and therapeutic target for reshaping immunosuppressive microenvironment.
PMID: 40993727
J Transl Med · 2025
0.80
4
Expressions of SH3BP5, LMO3, and SNAP25 in diffuse large B-cell lymphoma cells and their association with clinical features.
PMID: 27184832
Cancer Med · 2016
0.70
5
Novel blood-based hypomethylation of SH3BP5 is associated with very early-stage lung adenocarcinoma.
PMID: 34783986
Genes Genomics · 2022
0.60