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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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HOOK3
hook microtubule tethering protein 3
Chromosome 8 · 8p11.21
NCBI Gene: 84376Ensembl: ENSG00000168172.10HGNC: HGNC:23576UniProt: Q86VS8
59PubMed Papers
20Diseases
0Drugs
1Pathogenic Variants
FUNCTIONAL ROLE
Transporter
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
HOPS complexprotein bindingmicrotubule bindingdynactin bindingThyroid Gland Oncocytic Follicular Carcinomathyroid gland oncocytic adenomaShort staturepapillary thyroid carcinoma
✦AI Summary

HOOK3 (hook microtubule tethering protein 3) serves as a critical scaffold protein that coordinates bidirectional cargo transport by linking opposite-polarity microtubule motors dynein and kinesin-3 KIF1C 1. The protein functions as a cargo adaptor that binds to and activates cytoplasmic dynein-1/dynactin complex while simultaneously interacting with KIF1C through its tail region, enabling dynein to transport KIF1C toward microtubule minus ends and KIF1C to transport dynein toward plus ends 1. HOOK3 forms part of the FHF complex with FTS and FHIP proteins, which exists in an autoinhibited state that KIF1C can relieve to activate cargo transport 2. The protein also activates KIF1C by relieving its autoinhibitory interactions, working alongside PTPN21 to enable cargo-activated transport 3. HOOK3 demonstrates significant disease relevance, with reduced expression observed in Alzheimer's disease leading to slowed endosomal transport and increased β-amyloid production 4. In cancer, HOOK3 exhibits tumor-suppressive properties in gastric cancer by regulating the SP1/VEGFA pathway 5, while amplification in bladder urothelial carcinoma correlates with improved patient survival 6. These findings highlight HOOK3's dual role in maintaining cellular transport homeostasis and its potential therapeutic significance in neurodegenerative diseases and cancer.

Sources cited
1
HOOK3 scaffolds dynein/dynactin and KIF1C motors for bidirectional transport
PMID: 31320392
2
HOOK3 forms FHF complex that KIF1C can activate
PMID: 39747486
3
HOOK3 relieves KIF1C autoinhibition along with PTPN21
PMID: 31217419
4
HOOK3 expression is reduced in Alzheimer's disease and affects β-amyloid production
PMID: 25799409
5
HOOK3 suppresses gastric cancer via SP1/VEGFA pathway
PMID: 38228617
6
HOOK3 amplification correlates with improved survival in bladder cancer
PMID: 39626914
Disease Associationsⓘ20
thyroid gland oncocytic adenomaOpen Targets
0.37Weak
Thyroid Gland Oncocytic Follicular CarcinomaOpen Targets
0.37Weak
Short statureOpen Targets
0.32Weak
papillary thyroid carcinomaOpen Targets
0.28Weak
Poorly Differentiated Thyroid Gland CarcinomaOpen Targets
0.28Weak
myocardial infarctionOpen Targets
0.27Weak
Alzheimer diseaseOpen Targets
0.24Weak
Abruptio PlacentaeOpen Targets
0.23Weak
bladder transitional cell carcinomaOpen Targets
0.19Weak
acute lymphoblastic leukemiaOpen Targets
0.19Weak
lung adenocarcinomaOpen Targets
0.19Weak
pancreatic ductal adenocarcinomaOpen Targets
0.19Weak
bile duct carcinomaOpen Targets
0.18Weak
brain glioblastomaOpen Targets
0.18Weak
extranodal nasal NK/T cell lymphomaOpen Targets
0.18Weak
gastric intestinal type adenocarcinomaOpen Targets
0.18Weak
Hepatobiliary NeoplasmOpen Targets
0.18Weak
rectal adenocarcinomaOpen Targets
0.18Weak
superficial spreading melanomaOpen Targets
0.18Weak
urinary bladder carcinomaOpen Targets
0.18Weak
Pathogenic Variants1
NM_032410.4(HOOK3):c.225T>G (p.Asn75Lys)Likely pathogenic
Short stature
☆☆☆☆2001→ Residue 75
View on ClinVar ↗
Related Genes
DCTN1Protein interaction100%BICD1Protein interaction99%HOOK1Protein interaction90%HOOK2Protein interaction87%NINProtein interaction78%AKTIPProtein interaction77%
Tissue Expression6 tissues
Brain
100%
Lung
59%
Heart
52%
Liver
48%
Ovary
43%
Bone Marrow
40%
Gene Interaction Network
Click a node to explore
HOOK3DCTN1BICD1HOOK1HOOK2NINAKTIP
PROTEIN STRUCTURE
Preparing viewer…
PDB6B9H · 1.50 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.39Moderately Constrained
pLIⓘ
1.00Intolerant
Observed/Expected LoF0.21 [0.13–0.39]
RankingsWhere HOOK3 stands among ~20K protein-coding genes
  • #7,777of 20,598
    Most Researched59
  • #5,051of 5,498
    Most Pathogenic Variants1
  • #1,883of 17,882
    Most Constrained (LOEUF)0.39 · top quartile
Genes detectedHOOK3
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
HOOK3 Amplification in Bladder Urothelial Carcinoma: Insights from Gene Expression and Survival Analysis.
PMID: 39626914
Anticancer Res · 2024
1.00
2
Molecular basis for assembly and activation of the Hook3 - KIF1C complex-dependent transport machinery.
PMID: 40312563
EMBO Rep · 2025
0.90
3
PTPN21 and Hook3 relieve KIF1C autoinhibition and activate intracellular transport.
PMID: 31217419
Nat Commun · 2019
0.80
4
HOOK3 suppresses proliferation and metastasis in gastric cancer via the SP1/VEGFA axis.
PMID: 38228617
Cell Death Discov · 2024
0.70
5
KIF1C activates and extends dynein movement through the FHF cargo adapter.
PMID: 39747486
Nat Struct Mol Biol · 2025
0.60