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6 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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RIMBP3
RIMS binding protein 3
Chromosome 22 · 22q11.21
NCBI Gene: 85376Ensembl: ENSG00000275793.1HGNC: HGNC:29344UniProt: Q9UFD9
18PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Highly Constrained
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingmanchettebenzodiazepine receptor bindingspermatid developmentazoospermiapartial chromosome Y deletionspermatogenic failure 78spermatogenic failure 20
✦AI Summary

RIMBP3 (RIMS binding protein 3) is a manchette-associated protein essential for male fertility through its role in sperm morphogenesis. During spermiogenesis, RIMBP3 localizes to the manchette, a transient microtubular structure critical for converting round spermatids into mature sperm 1. The protein functions as a component of a multiprotein complex involving HOOK family proteins, KLC3, and other cargo adapter proteins that regulate microtubule dynamics 2. RIMBP3 phosphorylation is regulated by HIPK4 kinase during spermiogenesis, with these phosphorylation events essential for proper sperm head shaping 3. Targeted deletion of RIMBP3 in mice causes male infertility characterized by abnormal sperm morphology, including deformed nuclei and detached acrosomes 1. RIMBP3 interacts directly with Hook1, a manchette protein required for proper sperm head morphogenesis, suggesting this interaction is critical for manchette function 1. In humans, RIMBP3 is significantly downregulated in non-obstructive azoospermia (NOA) and is among eight genes implicated in impaired spermatogenesis 4. These findings establish RIMBP3 as a key molecular component whose dysfunction contributes to male infertility.

Sources cited
1
RIMBP3 is a manchette-associated protein essential for spermiogenesis and sperm head morphogenesis; targeted deletion causes male infertility with abnormal sperm morphology
PMID: 19091768
2
RIMBP3 is part of a multiprotein complex with HOOK proteins and KLC3 required for manchette function and microtubule dynamics
PMID: 28003339
3
HIPK4 regulates phosphorylation of RIMBP3 during spermiogenesis, with phosphorylation essential for sperm head morphogenesis
PMID: 35931115
4
RIMBP3 is among eight genes downregulated in non-obstructive azoospermia and male infertility as a potential biomarker
PMID: 35173218
5
RIMBP3 is a mammalian-specific RIM-binding protein family member encoding three SH3 domains and fibronectin III repeats
PMID: 17855024
Disease Associationsⓘ20
azoospermiaOpen Targets
0.10Weak
partial chromosome Y deletionOpen Targets
0.08Suggestive
spermatogenic failure 78Open Targets
0.07Suggestive
spermatogenic failure 20Open Targets
0.07Suggestive
spermatogenic failure, X-linked, 3Open Targets
0.07Suggestive
spermatogenic failure 54Open Targets
0.07Suggestive
spermatogenic failure 40Open Targets
0.07Suggestive
spermatogenic failure 10Open Targets
0.07Suggestive
spermatogenic failure 11Open Targets
0.07Suggestive
spermatogenic failure 76Open Targets
0.07Suggestive
spermatogenic failure 80Open Targets
0.07Suggestive
spermatogenic failure 47Open Targets
0.07Suggestive
spermatogenic failure 65Open Targets
0.07Suggestive
spermatogenic failure 72Open Targets
0.07Suggestive
spermatogenic failure 39Open Targets
0.07Suggestive
spermatogenic failure 84Open Targets
0.07Suggestive
spermatogenic failure 93Open Targets
0.07Suggestive
spermatogenic failure 18Open Targets
0.07Suggestive
spermatogenic failure 27Open Targets
0.07Suggestive
spermatogenic failure 46Open Targets
0.07Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
HOOK1Protein interaction88%RIMBP3CProtein interaction88%RIMBP3BProtein interaction88%RIMS2Protein interaction81%KIF3BProtein interaction74%TSSK4Shared pathway50%
Tissue Expression6 tissues
Brain
100%
Ovary
9%
Lung
9%
Liver
0%
Heart
0%
Bone Marrow
0%
Gene Interaction Network
Click a node to explore
RIMBP3HOOK1RIMBP3CRIMBP3BRIMS2KIF3BTSSK4
PROTEIN STRUCTURE
Preparing viewer…
PDB2EGE · NMR
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.16Highly Constrained
pLIⓘ
1.00Intolerant
Observed/Expected LoF0.03 [0.01–0.16]
RankingsWhere RIMBP3 stands among ~20K protein-coding genes
  • #14,793of 20,598
    Most Researched18
  • #263of 17,882
    Most Constrained (LOEUF)0.16 · top 5%
Genes detectedRIMBP3
Sources retrieved6 papers
Response time—
📄 Sources
6▼
1
Using publicly available transcriptomic data to identify mechanistic and diagnostic biomarkers in azoospermia and overall male infertility.
PMID: 35173218
Sci Rep · 2022
1.00
2
Inhibitory activity of
PMID: 36744215
Front Pharmacol · 2022
0.83
3
LRGUK1 is part of a multiprotein complex required for manchette function and male fertility.
PMID: 28003339
FASEB J · 2017
0.67
4
Homeodomain-interacting protein kinase HIPK4 regulates phosphorylation of manchette protein RIMBP3 during spermiogenesis.
PMID: 35931115
J Biol Chem · 2022
0.50
5
Structure and evolution of RIM-BP genes: identification of a novel family member.
PMID: 17855024
Gene · 2007
0.33