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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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SPTBN5
spectrin beta, non-erythrocytic 5
Chromosome 15 · 15q15.1
NCBI Gene: 51332Ensembl: ENSG00000137877.10HGNC: HGNC:15680UniProt: Q9NRC6
29PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
lysosomal transportphotoreceptor disc membranephotoreceptor connecting ciliumGolgi organizationtype 2 diabetes mellitusModerate intellectual disabilityAutistic behaviorautism
✦AI Summary

SPTBN5 (spectrin beta, non-erythrocytic 5) encodes a beta-spectrin subunit that functions as a cytoskeletal scaffolding protein. Spectrins are assembled from α and β subunits and serve as common components of cytoskeletons, binding to cytoskeletal elements and the plasma membrane to allow proper localization of essential membrane proteins, signal transduction, and cellular scaffolding 1. The protein localizes to various cellular compartments including the cytoplasm, plasma membrane, and photoreceptor structures, where it binds actin filaments and participates in cytoskeletal organization based on GO annotations. SPTBN5 variants have been associated with neurodevelopmental disorders, with four different families showing missense and nonsense mutations causing intellectual disability ranging from mild to severe, aggressive tendencies, craniofacial dysmorphisms, autistic behavior, and gastroesophageal reflux 2. Additionally, SPTBN5 appears to play roles in diverse pathological processes including colorectal cancer progression, where downregulation significantly decreases cell proliferation, migration, and clonogenicity 3, and has been implicated in progressive supranuclear palsy through genome-wide association studies examining tau neuropathology 4. The gene has also been identified in studies of sacral agenesis 5 and shows altered methylation patterns in World Trade Center-exposed populations 6, suggesting broader roles in human disease beyond neurodevelopment.

Sources cited
1
Spectrins function as cytoskeletal scaffolding proteins binding to cytoskeletal elements and plasma membrane
PMID: 33847457
2
SPTBN5 variants cause neurodevelopmental disorders including intellectual disability and autistic behavior
PMID: 35782384
3
SPTBN5 downregulation decreases colorectal cancer cell proliferation, migration, and clonogenicity
PMID: 39692974
4
SPTBN5 is associated with tau neuropathology in progressive supranuclear palsy
PMID: 33635380
5
SPTBN5 variants identified in patients with sacral agenesis
PMID: 35274497
6
SPTBN5 shows altered DNA methylation in World Trade Center-exposed populations
PMID: 32751422
Disease Associationsⓘ20
type 2 diabetes mellitusOpen Targets
0.21Weak
autismOpen Targets
0.15Weak
Autistic behaviorOpen Targets
0.15Weak
Delayed speech and language developmentOpen Targets
0.15Weak
Moderate intellectual disabilityOpen Targets
0.15Weak
premature birthOpen Targets
0.15Weak
hypertensionOpen Targets
0.10Suggestive
Increased blood pressureOpen Targets
0.07Suggestive
Abnormality of the skeletal systemOpen Targets
0.07Suggestive
deafnessOpen Targets
0.06Suggestive
autosomal dominant nonsyndromic hearing lossOpen Targets
0.05Suggestive
autosomal recessive nonsyndromic hearing loss 9Open Targets
0.05Suggestive
schwannomaOpen Targets
0.04Suggestive
schwannomatosisOpen Targets
0.04Suggestive
SMARCB1-related schwannomatosisOpen Targets
0.04Suggestive
hearing loss, autosomal recessiveOpen Targets
0.04Suggestive
Autosomal recessive intermediate Charcot-Marie-Tooth disease type BOpen Targets
0.03Suggestive
Charcot-Marie-Tooth disease recessive intermediate BOpen Targets
0.03Suggestive
Charcot-Marie-Tooth disease type 4B1Open Targets
0.03Suggestive
atrial fibrillationOpen Targets
0.03Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
ANK1Protein interaction96%ANK2Protein interaction96%ANK3Protein interaction96%EPB41Protein interaction96%SPTA1Protein interaction96%SPTAN1Protein interaction96%
Tissue Expression6 tissues
Liver
100%
Bone Marrow
72%
Lung
62%
Heart
56%
Ovary
54%
Brain
6%
Gene Interaction Network
Click a node to explore
SPTBN5ANK1ANK2ANK3EPB41SPTA1SPTAN1
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt Q9NRC6
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
1.36LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF1.26 [1.17–1.36]
RankingsWhere SPTBN5 stands among ~20K protein-coding genes
  • #12,242of 20,598
    Most Researched29
  • #14,198of 17,882
    Most Constrained (LOEUF)1.36
Genes detectedSPTBN5
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Heterozygous variants in SPTBN1 cause intellectual disability and autism.
PMID: 33847457
Am J Med Genet A · 2021
1.00
2
PMID: 35782384
Front Mol Neurosci · 2022
0.90
3
Identification of a pyroptosis-related prognostic model for colorectal cancer and validation of the core gene SPTBN5.
PMID: 39692974
Discov Oncol · 2024
0.80
4
Latent trait modeling of tau neuropathology in progressive supranuclear palsy.
PMID: 33635380
Acta Neuropathol · 2021
0.70
5
Exome sequencing identifies variants in infants with sacral agenesis.
PMID: 35274497
Birth Defects Res · 2022
0.60