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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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KIAA0319L
KIAA0319 like
Chromosome 1 · 1p34.3
NCBI Gene: 79932Ensembl: ENSG00000142687.19HGNC: HGNC:30071UniProt: A0A8V8TKL4
50PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingGolgi apparatuscytoplasmic vesicleplasma membranesystemic lupus erythematosussystemic sclerodermaazoospermiapartial chromosome Y deletion
✦AI Summary

KIAA0319L encodes a type I transmembrane protein that serves as a universal receptor for adeno-associated virus (AAV) infection across multiple serotypes 1. The protein facilitates rapid endocytosis from the plasma membrane and trafficking to the trans-Golgi network, directly binding AAV particles to enable viral entry 1. KIAA0319L interacts with the FAM91A1-TBC1D23 complex to regulate endosome-to-Golgi trafficking, which is crucial for axonal growth and neuronal development 2. Genetic ablation of KIAA0319L renders mammalian cells and mice highly resistant to AAV infection, highlighting its essential role in viral gene therapy vectors 1. The gene has clinical significance in multiple diseases: it serves as a susceptibility locus for autoimmune conditions including systemic sclerosis and systemic lupus erythematosus, showing overexpression in patient blood cells 3. KIAA0319L is also implicated in developmental dyslexia, with in utero knockdown experiments causing neuronal migration defects and periventricular heterotopia formation 4. Additionally, knockout mice lacking KIAA0319L display auditory processing deficits and abnormal brainstem responses, suggesting roles in auditory system function 5. Some studies have explored associations with recurrent pregnancy loss, though results require further validation 6.

Sources cited
1
KIAA0319L acts as a universal AAV receptor enabling viral entry through endocytosis and trafficking to trans-Golgi network
PMID: 26814968
2
KIAA0319L interacts with FAM91A1-TBC1D23 complex to regulate endosome-to-Golgi trafficking important for axonal growth
PMID: 37903274
3
KIAA0319L is a susceptibility locus for systemic sclerosis and lupus, with overexpression in patient blood cells
PMID: 23740937
4
Knockdown of KIAA0319L during rat corticogenesis causes neuronal migration defects and periventricular heterotopia
PMID: 23831424
5
KIAA0319L knockout mice show auditory processing deficits and abnormal brainstem responses
PMID: 29045729
6
Potential association between KIAA0319L polymorphisms and recurrent pregnancy loss investigated
PMID: 25596907
Disease Associationsⓘ20
systemic lupus erythematosusOpen Targets
0.19Weak
systemic sclerodermaOpen Targets
0.18Weak
azoospermiaOpen Targets
0.11Weak
partial chromosome Y deletionOpen Targets
0.07Suggestive
spermatogenic failure 78Open Targets
0.07Suggestive
spermatogenic failure 91Open Targets
0.07Suggestive
spermatogenic failure, X-linked, 7Open Targets
0.07Suggestive
spermatogenic failure 85Open Targets
0.07Suggestive
spermatogenic failure 54Open Targets
0.07Suggestive
spermatogenic failure 65Open Targets
0.07Suggestive
spermatogenic failure 10Open Targets
0.07Suggestive
spermatogenic failure 11Open Targets
0.07Suggestive
spermatogenic failure 84Open Targets
0.07Suggestive
spermatogenic failure 93Open Targets
0.07Suggestive
spermatogenic failure, X-linked, 8Open Targets
0.07Suggestive
spermatogenic failure 39Open Targets
0.07Suggestive
spermatogenic failure 56Open Targets
0.07Suggestive
spermatogenic failure 92Open Targets
0.07Suggestive
spermatogenic failure 94Open Targets
0.07Suggestive
spermatogenic failure, X-linked, 3Open Targets
0.07Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
CEP85LShared pathway100%RTN4RProtein interaction87%ASTN1Shared pathway50%ASTN2Shared pathway50%POMGNT2Shared pathway33%ACAP3Shared pathway33%
Tissue Expression6 tissues
Heart
100%
Liver
70%
Lung
60%
Brain
54%
Ovary
43%
Bone Marrow
33%
Gene Interaction Network
Click a node to explore
KIAA0319LCEP85LRTN4RASTN1ASTN2POMGNT2ACAP3
PROTEIN STRUCTURE
Preparing viewer…
PDB7KPN · 1.90 Å · EM
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.61LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.48 [0.38–0.61]
RankingsWhere KIAA0319L stands among ~20K protein-coding genes
  • #8,833of 20,598
    Most Researched50
  • #4,236of 17,882
    Most Constrained (LOEUF)0.61 · top quartile
Genes detectedKIAA0319L
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
An essential receptor for adeno-associated virus infection.
PMID: 26814968
Nature · 2016
1.00
2
An alternate receptor for adeno-associated viruses.
PMID: 40664211
Cell · 2025
0.90
3
FAM91A1-TBC1D23 complex structure reveals human genetic variations susceptible for PCH.
PMID: 37903274
Proc Natl Acad Sci U S A · 2023
0.80
4
Association between KIAA0319L, PXK and JAZF1 gene polymorphisms and unexplained recurrent pregnancy loss in Chinese Han couples.
PMID: 25596907
Reprod Biomed Online · 2015
0.70
5
Knockout Mice for Dyslexia Susceptibility Gene Homologs KIAA0319 and KIAA0319L have Unaffected Neuronal Migration but Display Abnormal Auditory Processing.
PMID: 29045729
Cereb Cortex · 2017
0.60