TPGS2

Chr 18

tubulin polyglutamylase complex subunit 2

This protein is a subunit of the tubulin polyglutamylase complex that mediates polyglutamylation of cilia and flagella, which is essential for their biogenesis and motility. Mutations cause spastic paraplegia 57, an autosomal recessive condition characterized by progressive spasticity primarily affecting the lower limbs. The gene is highly constrained against loss-of-function variants, indicating that complete loss of protein function is likely not tolerated.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 1.06
Clinical SummaryTPGS2
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
Some data sources returned errors (1)

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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.06LOEUF
pLI 0.000
Z-score 1.40
OE 0.59 (0.341.06)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.03Z-score
OE missense 1.01 (0.891.14)
165 obs / 164.0 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.59 (0.341.06)
00.351.4
Missense OE1.01 (0.891.14)
00.61.4
Synonymous OE0.87
01.21.6
LoF obs/exp: 8 / 13.6Missense obs/exp: 165 / 164.0Syn Z: 0.82

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

TPGS2 · 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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