VPS13B

Chr 8AR

vacuolar protein sorting 13 homolog B

Also known as: BLTP5B, CHS1, COH1

The protein mediates lipid transfer between membranes at organelle contact sites and functions as a tethering factor in endocytic recycling pathways, with roles in Golgi assembly and nervous system development. Mutations cause Cohen syndrome, a multisystem disorder affecting the eye, hematological system, and central nervous system. Inheritance is autosomal recessive, and the gene shows high constraint against loss-of-function variants.

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismARLOEUF 0.651 OMIM phenotype
Clinical SummaryVPS13B
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Gene-Disease Validity (ClinGen)
Cohen syndrome · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
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Clinical Trials
2 active or recruiting trials — potential therapeutic options may be available

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.65LOEUF
pLI 0.000
Z-score 5.71
OE 0.55 (0.470.65)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.98Z-score
OE missense 0.94 (0.900.97)
1947 obs / 2072.8 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.55 (0.470.65)
00.351.4
Missense OE0.94 (0.900.97)
00.61.4
Synonymous OE1.03
01.21.6
LoF obs/exp: 105 / 189.9Missense obs/exp: 1947 / 2072.8Syn Z: -0.63

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

VPS13B · protein map & ClinVar variants

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

3D Protein StructureAlphaFold
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 ↗