VPS50

Chr 7AR

VPS50 subunit of EARP/GARPII complex

Also known as: CCDC132, NEDMSC, VPS54L

The VPS50 protein is a component of the EARP complex that mediates endocytic recycling by tethering the complex to recycling endosomes and promoting recycling of internalized receptors like transferrin receptor to the plasma membrane. Biallelic mutations cause a neurodevelopmental disorder with microcephaly, seizures, and neonatal cholestasis, reflecting involvement of the nervous system, brain development, and hepatic function. This condition follows autosomal recessive inheritance.

OMIMResearchSummary from RefSeq, OMIM, UniProt
DNmechanismARLOEUF 0.331 OMIM phenotype
Clinical SummaryVPS50
Population Constraint (gnomAD)
Moderately constrained gene (pLI 0.81) — some intolerance to loss-of-function variants.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.33LOEUF
pLI 0.814
Z-score 5.63
OE 0.21 (0.130.33)
Highly constrained

Highly LoF-intolerant (top ~10% of genes)

Missense Constraint
2.50Z-score
OE missense 0.68 (0.620.74)
325 obs / 479.1 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios
LoF OE0.21 (0.130.33)
00.351.4
Missense OE0.68 (0.620.74)
00.61.4
Synonymous OE1.02
01.21.6
LoF obs/exp: 12 / 58.4Missense obs/exp: 325 / 479.1Syn Z: -0.25
DN
0.6357th %ile
GOF
0.6150th %ile
LOF
0.3357th %ile

The highest-scoring mechanism for this gene is dominant-negative.

DNprediction above median

Note: In-silico variant effect predictors (SIFT, PolyPhen, REVEL, CADD) may underestimate pathogenicity of missense variants in genes with GOF or DN mechanisms. Consider functional evidence and clinical context.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

VPS50 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

3D Protein StructureAlphaFold

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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Clinical Literature
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Full-Text Mentions
NLP-detected gene mentions in article bodies · via PubTator3
PubTator3
Top 5 full-text resultsSearch PubTator3 ↗