DPH7

Chr 9

diphthamide biosynthesis 7

The protein catalyzes the demethylation of diphthine methyl ester to form diphthine, an intermediate in diphthamide biosynthesis, which is a post-translational modification of translation elongation factor 2. Mutations cause autosomal recessive developmental and epileptic encephalopathy with onset in early infancy, characterized by severe intellectual disability, seizures, and developmental delays. This represents a disorder of protein synthesis affecting the central nervous system.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 1.22
Clinical SummaryDPH7
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
1.22LOEUF
pLI 0.000
Z-score 0.87
OE 0.78 (0.511.22)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.00Z-score
OE missense 1.00 (0.901.11)
255 obs / 255.0 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.78 (0.511.22)
00.351.4
Missense OE1.00 (0.901.11)
00.61.4
Synonymous OE1.06
01.21.6
LoF obs/exp: 14 / 18.0Missense obs/exp: 255 / 255.0Syn Z: -0.50

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

DPH7 · 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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