GTPBP8

Chr 3

GTP binding protein 8

Also known as: HSPC135, MRX8

GTPBP8 encodes a mitochondrial GTPase that binds GTP and metal ions. Mutations cause autosomal recessive developmental and epileptic encephalopathy with microcephaly, typically presenting in infancy. The gene is highly constrained against loss-of-function variants, indicating its critical importance for normal development.

OMIMResearchSummary from RefSeq
LOEUF 1.51
Clinical SummaryGTPBP8
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.51LOEUF
pLI 0.000
Z-score 0.26
OE 0.92 (0.581.51)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.56Z-score
OE missense 1.13 (1.001.28)
173 obs / 153.3 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.92 (0.581.51)
00.351.4
Missense OE1.13 (1.001.28)
00.61.4
Synonymous OE1.08
01.21.6
LoF obs/exp: 11 / 12.0Missense obs/exp: 173 / 153.3Syn Z: -0.48

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

GTPBP8 · 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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