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GeneE
10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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SLIRP
SRA stem-loop interacting RNA binding protein
Chromosome 14 · 14q24.3
NCBI Gene: 81892Ensembl: ENSG00000119705.10HGNC: HGNC:20495UniProt: A0A087WUN7
126PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Transcription Factor
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
RNA bindingmRNA bindingprotein bindingmitochondrionAbnormality of the genital systemneurodegenerative diseasecongenital lactic acidosis, Saguenay-Lac-Saint-Jean typeazoospermia
✦AI Summary

SLIRP is a mitochondrial RNA-binding protein that functions primarily as a post-transcriptional regulator of mitochondrial gene expression. As part of a ribonucleoprotein complex with LRPPRC, SLIRP stabilizes mitochondrial mRNAs and facilitates their delivery to mitoribosomes for translation 12. The LRPPRC-SLIRP complex directly binds mRNAs and associates with mitoribosomal proteins to form a corridor for mRNA handoff, with transcript-specific effects on translation efficiency, particularly affecting cytochrome c oxidase subunits 2. Beyond mitochondrial function, SLIRP acts as a nuclear receptor corepressor, interacting with androgen receptor in an androgen- and kinase-regulated manner to modulate transcription of AR target genes 3. In skeletal muscle, SLIRP regulates mitochondrial structure and respiration as a PGC-1α target, with exercise training increasing SLIRP expression across sexes, though less prominently in type 2 diabetes 4. Pathologically, mutations in SLIRP cause devastating mitochondrial diseases including Leigh syndrome characterized by COX deficiency 1. Recently, SLIRP was identified as an amplifier of antiviral signaling through positive feedback regulation of mitochondrial dsRNA-triggered responses, contributing to autoimmune disease pathogenesis 5. SLIRP loss also reduces sperm motility and increases oxidative stress, affecting male fertility 6.

Sources cited
1
SLIRP interacts with LRPPRC in ribonucleoprotein complexes regulating mitochondrial mRNA stability and protein synthesis
PMID: 20200222
2
Structural evidence that LRPPRC-SLIRP complex delivers mRNAs to mitoribosomes with transcript-specific effects on translation
PMID: 39134711
3
SLIRP is a PGC-1α target regulating mitochondrial structure and respiration in skeletal muscle; exercise increases SLIRP expression
PMID: 39537626
4
SLIRP functions as a nuclear receptor corepressor of androgen receptor in an androgen- and kinase-regulated manner
PMID: 31819114
5
SLIRP amplifies antiviral signaling via positive feedback regulation of mitochondrial dsRNAs and contributes to autoimmune diseases
PMID: 40253699
6
SLIRP regulates sperm motility and oxidative stress; SLIRP loss reduces fertility and increases ROS
PMID: 33150185
7
LRPPRC-SLIRP complex is critical for maintaining complex I levels and coordinates tissue-specific mitochondrial translation
PMID: 39087558
Disease Associationsⓘ20
Abnormality of the genital systemOpen Targets
0.27Weak
neurodegenerative diseaseOpen Targets
0.20Weak
congenital lactic acidosis, Saguenay-Lac-Saint-Jean typeOpen Targets
0.12Weak
azoospermiaOpen Targets
0.09Suggestive
spermatogenic failure, X-linked, 5Open Targets
0.06Suggestive
spermatogenic failure 65Open Targets
0.06Suggestive
spermatogenic failure 84Open Targets
0.06Suggestive
spermatogenic failure 93Open Targets
0.06Suggestive
spermatogenic failure 72Open Targets
0.06Suggestive
spermatogenic failure 56Open Targets
0.06Suggestive
spermatogenic failure 92Open Targets
0.06Suggestive
spermatogenic failure 94Open Targets
0.06Suggestive
spermatogenic failure 18Open Targets
0.06Suggestive
spermatogenic failure 27Open Targets
0.06Suggestive
spermatogenic failure 46Open Targets
0.06Suggestive
spermatogenic failure 51Open Targets
0.06Suggestive
spermatogenic failure 42Open Targets
0.06Suggestive
spermatogenic failure, X-linked, 3Open Targets
0.06Suggestive
spermatogenic failure 43Open Targets
0.06Suggestive
spermatogenic failure 49Open Targets
0.06Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
NDUFB2Protein interaction100%SNW1Protein interaction100%UQCRQProtein interaction100%TOMM7Protein interaction75%GRSF1Protein interaction74%LRPPRCProtein interaction74%
Tissue Expression6 tissues
Heart
100%
Liver
90%
Brain
74%
Bone Marrow
62%
Ovary
39%
Lung
38%
Gene Interaction Network
Click a node to explore
SLIRPNDUFB2SNW1UQCRQTOMM7GRSF1LRPPRC
PROTEIN STRUCTURE
Preparing viewer…
PDB8ANY · 2.85 Å · EM
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
1.42LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.88 [0.56–1.42]
RankingsWhere SLIRP stands among ~20K protein-coding genes
  • #3,745of 20,598
    Most Researched126 · top quartile
  • #14,661of 17,882
    Most Constrained (LOEUF)1.42
Genes detectedSLIRP
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
The mitochondrial mRNA-stabilizing protein SLIRP regulates skeletal muscle mitochondrial structure and respiration by exercise-recoverable mechanisms.
PMID: 39537626
Nat Commun · 2024
1.00
2
LRPPRC and SLIRP synergize to maintain sufficient and orderly mammalian mitochondrial translation.
PMID: 39087558
Nucleic Acids Res · 2024
0.90
3
Structural basis of LRPPRC-SLIRP-dependent translation by the mitoribosome.
PMID: 39134711
Nat Struct Mol Biol · 2024
0.80
4
SLIRP amplifies antiviral signaling via positive feedback regulation and contributes to autoimmune diseases.
PMID: 40253699
Cell Rep · 2025
0.70
5
Interaction between androgen receptor and coregulator SLIRP is regulated by Ack1 tyrosine kinase and androgen.
PMID: 31819114
Sci Rep · 2019
0.60