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GeneE
10 sources retrieved Β· Most recent: April 2026 Β· Index updated 14 days ago
β“˜GeneE is for informational purposes only. It is not a substitute for professional medical advice, diagnosis, or treatment.
WASHC5
WASH complex subunit 5
Chromosome 8 Β· 8q24.13
NCBI Gene: 9897Ensembl: ENSG00000164961.16HGNC: HGNC:28984UniProt: E7EQI7
89PubMed Papers
22Diseases
0Drugs
42Pathogenic Variants
FUNCTIONAL ROLE
Transporter
CLINICAL
OMIM Disease Gene
DATA QUALITY
βœ“ Experimental GO Evidenceβœ“ Swiss-Prot Reviewed
early endosomeprotein bindingcytosolWASH complexhereditary spastic paraplegia 8Autosomal dominant spastic paraplegia type 8Ritscher-Schinzel syndrome 13C syndrome
✦AI Summary

WASHC5 encodes a core component of the WASH complex, a nucleation-promoting factor that recruits and activates the Arp2/3 complex to induce actin polymerization at endosomal membranes 12. This activity is essential for endosome fission and tubulation during endosomal sorting and trafficking 3. WASHC5 also regulates protein recycling through CAV1-dependent mechanisms and maintains integrin-mediated cell adhesion 3. Pathogenic WASHC5 variants cause two distinct genetic disorders. Autosomal recessive mutations cause Ritscher-Schinzel syndrome 1 (RTSCS1), characterized by craniofacial dysmorphism, macrocephaly, developmental delay, intellectual disability, ataxic gait, and cardiovascular/cerebellar malformations 45. Autosomal dominant mutations cause hereditary spastic paraplegia type SPG8 (SPG8), presenting with progressive leg spasticity and weakness with variable age of onset 67. SPG8-associated mutations impair endosomal fission and CAV1-dependent cell adhesion 3. WASHC5 functions within a broader trafficking network coordinating with the CCC and Retriever complexes 8. Disease mechanisms involve disrupted endosomal homeostasis and abnormal ER function affecting skeletal, cardiac, and nervous system development 5. The WASH complex's role in maintaining protein abundance through inhibition of lysosomal degradation appears critical for cellular pathogenesis 3.

Sources cited
1
WASHC5 is a nucleation-promoting factor component of the WASH complex that recruits Arp2/3 complex
PMID: 19922875
2
WASH complex activates Arp2/3 complex to induce actin polymerization at endosomes for fission of transport tubules
PMID: 20498093
3
WASHC5 mutations in SPG8 impair endosomal fission, CAV1 stability, and integrin-mediated cell adhesion through effects on lysosomal degradation
PMID: 31911435
4
Autosomal recessive WASHC5 variants cause Ritscher-Schinzel syndrome 1 with craniofacial dysmorphism, developmental delay, intellectual disability, and ataxia
PMID: 36130690
5
WASHC5 knockout in zebrafish causes maxillofacial, cardiac, and nervous system defects with disrupted ER homeostasis and altered expression of developmental genes
PMID: 39988189
6
Autosomal dominant WASHC5 mutations cause spastic paraplegia type SPG8 with variable presentation
PMID: 34184482
7
Novel WASHC5 variant identified in childhood-onset hereditary spastic paraplegia patient with progressive leg spasticity
PMID: 38301322
8
WASHC5 functions within a network with WASH, CCC, and Retriever complexes coordinating endosomal protein recycling and membrane trafficking
PMID: 40448120
Disease Associationsβ“˜22
hereditary spastic paraplegia 8Open Targets
0.80Strong
Autosomal dominant spastic paraplegia type 8Open Targets
0.76Strong
Ritscher-Schinzel syndrome 1Open Targets
0.73Strong
3C syndromeOpen Targets
0.63Moderate
Ritscher-Schinzel syndromeOpen Targets
0.59Moderate
genetic disorderOpen Targets
0.49Moderate
hereditary spastic paraplegiaOpen Targets
0.35Weak
Lower limb spasticityOpen Targets
0.26Weak
Spastic paraplegiaOpen Targets
0.16Weak
autosomal dominant complex spastic paraplegiaOpen Targets
0.15Weak
Muscle weaknessOpen Targets
0.15Weak
Intellectual disabilityOpen Targets
0.12Weak
Equinovarus deformityOpen Targets
0.12Weak
HeadacheOpen Targets
0.12Weak
Loss of ambulationOpen Targets
0.12Weak
Severe intellectual disability and progressive spastic paraplegiaOpen Targets
0.12Weak
Spastic gaitOpen Targets
0.12Weak
Stress urinary incontinenceOpen Targets
0.12Weak
atrial fibrillationOpen Targets
0.12Weak
SpasticityOpen Targets
0.11Weak
Ritscher-Schinzel syndrome 1UniProt
Spastic paraplegia 8, autosomal dominantUniProt
Pathogenic Variants42
NM_014846.4(WASHC5):c.1857G>C (p.Leu619Phe)Pathogenic
Hereditary spastic paraplegia 8|Ritscher-Schinzel syndrome;Hereditary spastic paraplegia 8
β˜…β˜…β˜†β˜†2025β†’ Residue 619
NM_014846.4(WASHC5):c.2086G>A (p.Gly696Ser)Pathogenic
not provided|Hereditary spastic paraplegia 8|Hereditary spastic paraplegia 8;Ritscher-Schinzel syndrome
β˜…β˜…β˜†β˜†2025β†’ Residue 696
NM_014846.4(WASHC5):c.1772C>T (p.Ser591Phe)Pathogenic
Hereditary spastic paraplegia 8|Hereditary spastic paraplegia 8;Ritscher-Schinzel syndrome|not provided
β˜…β˜…β˜†β˜†2024β†’ Residue 591
NM_014846.4(WASHC5):c.1876G>T (p.Val626Phe)Pathogenic
Hereditary spastic paraplegia 8|Hereditary spastic paraplegia|Ritscher-Schinzel syndrome;Hereditary spastic paraplegia 8|not provided|Inborn genetic diseases
β˜…β˜…β˜†β˜†2024β†’ Residue 626
NM_014846.4(WASHC5):c.2087G>A (p.Gly696Asp)Pathogenic
Hereditary spastic paraplegia 8|Ritscher-Schinzel syndrome;Hereditary spastic paraplegia 8
β˜…β˜…β˜†β˜†2023β†’ Residue 696
NM_014846.4(WASHC5):c.1771T>C (p.Ser591Pro)Pathogenic
Ritscher-Schinzel syndrome;Hereditary spastic paraplegia 8|Hereditary spastic paraplegia 8|Ritscher-Schinzel syndrome 1
β˜…β˜…β˜†β˜†2021β†’ Residue 591
NM_014846.4(WASHC5):c.3335+2T>ALikely pathogenic
Ritscher-Schinzel syndrome 1|WASHC5-related disorder|not provided
β˜…β˜†β˜†β˜†2026
NM_014846.4(WASHC5):c.186+1G>CLikely pathogenic
Hereditary spastic paraplegia 8;Ritscher-Schinzel syndrome
β˜…β˜†β˜†β˜†2025
NM_014846.4(WASHC5):c.2016+1G>ALikely pathogenic
Ritscher-Schinzel syndrome 1
β˜…β˜†β˜†β˜†2025
NM_014846.4(WASHC5):c.1443_1447del (p.Lys481fs)Pathogenic
Ritscher-Schinzel syndrome;Hereditary spastic paraplegia 8
β˜…β˜†β˜†β˜†2025β†’ Residue 481
NM_014846.4(WASHC5):c.2771-2A>GLikely pathogenic
Ritscher-Schinzel syndrome;Hereditary spastic paraplegia 8
β˜…β˜†β˜†β˜†2025
NM_014846.4(WASHC5):c.3163C>T (p.Gln1055Ter)Pathogenic
Ritscher-Schinzel syndrome;Hereditary spastic paraplegia 8
β˜…β˜†β˜†β˜†2025β†’ Residue 1055
NM_014846.4(WASHC5):c.3182-2A>GLikely pathogenic
Ritscher-Schinzel syndrome 1
β˜…β˜†β˜†β˜†2024
NM_014846.4(WASHC5):c.210del (p.Lys70fs)Pathogenic
Ritscher-Schinzel syndrome 1
β˜…β˜†β˜†β˜†2024β†’ Residue 70
NM_014846.4(WASHC5):c.1424G>A (p.Trp475Ter)Pathogenic
Hereditary spastic paraplegia 8;Ritscher-Schinzel syndrome
β˜…β˜†β˜†β˜†2024β†’ Residue 475
NM_014846.4(WASHC5):c.511C>T (p.Arg171Ter)Pathogenic
Ritscher-Schinzel syndrome;Hereditary spastic paraplegia 8
β˜…β˜†β˜†β˜†2024β†’ Residue 171
NM_014846.4(WASHC5):c.2505-1G>CLikely pathogenic
Hereditary spastic paraplegia 8;Ritscher-Schinzel syndrome
β˜…β˜†β˜†β˜†2024
NM_014846.4(WASHC5):c.3024_3025del (p.Leu1009fs)Pathogenic
Ritscher-Schinzel syndrome;Hereditary spastic paraplegia 8
β˜…β˜†β˜†β˜†2024β†’ Residue 1009
NM_014846.4(WASHC5):c.2595_2599del (p.Gln865fs)Pathogenic
Hereditary spastic paraplegia 8;Ritscher-Schinzel syndrome
β˜…β˜†β˜†β˜†2024β†’ Residue 865
NM_014846.4(WASHC5):c.711+1G>ALikely pathogenic
Hereditary spastic paraplegia 8|Lung cancer
β˜…β˜†β˜†β˜†2024
View on ClinVar β†—
Related Genes
CAPZA1Protein interaction100%CAPZBProtein interaction100%SPG11Protein interaction99%CAPZA2Protein interaction98%ZFYVE27Protein interaction88%NIPA1Protein interaction87%
Tissue Expression6 tissues
Heart
100%
Bone Marrow
92%
Brain
81%
Lung
69%
Ovary
56%
Liver
44%
Gene Interaction Network
Click a node to explore
WASHC5CAPZA1CAPZBSPG11CAPZA2ZFYVE27NIPA1
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted Β· UniProt Q12768
View on AlphaFold β†—
Constraintβ“˜
LOEUFβ“˜
0.79LoF Tolerant
pLIβ“˜
0.00Tolerant
Observed/Expected LoF0.65 [0.53–0.79]
RankingsWhere WASHC5 stands among ~20K protein-coding genes
  • #5,393of 20,598
    Most Researched89
  • #1,489of 5,498
    Most Pathogenic Variants42
  • #6,540of 17,882
    Most Constrained (LOEUF)0.79
Genes detectedWASHC5
Sources retrieved10 papers
Response timeβ€”
πŸ“„ Sources
10β–Ό
1
Expanding the pre- and postnatal phenotype of WASHC5 and CCDC22 -related Ritscher-Schinzel syndromes.
PMID: 36130690
Eur J Med Genet Β· 2022
1.00
2
Clinical and molecular characteristics of 26 fetuses with lethal multiple congenital contractures.
PMID: 38278647
Clin Genet Β· 2024
0.90
3
[Autosomal dominant spastic paraplegias].
PMID: 34184482
Zh Nevrol Psikhiatr Im S S Korsakova Β· 2021
0.80
4
Disrupted maxillofacial, cardiovascular, and nervous development in washc5 knockout Zebrafish: Insights into 3C syndrome.
PMID: 39988189
Gene Β· 2025
0.70
5
A Retrospective Review of 18 Patients With Childhood-Onset Hereditary Spastic Paraplegia, Nine With Novel Variants.
PMID: 38301322
Pediatr Neurol Β· 2024
0.60