DPH1

Chr 17

diphthamide biosynthesis 1

Also known as: DEDSSH, DPH2L, DPH2L1, OVCA1

The protein encoded by this gene is an enzyme involved in the biosynthesis of diphthamide, a modified histidine found only in elongation factor-2 (EEF2). Diphthamide residues in EEF2 are targeted for ADP-ribosylation by diphtheria toxin and Pseudomonas exotoxin A. Defects in this gene have been associated with both ovarian cancer and autosomal recessive intellectual disability with short stature, craniofacial, and ectodermal anomalies. [provided by RefSeq, Oct 2016]

GeneReviewsResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtDevelopmental delay with short stature, dysmorphic facial features, and sparse hair 1

Clinical highlights

Gene-disease validity (ClinGen)
developmental delay with short stature, dysmorphic facial features, and sparse hair · ARDefinitivesufficient evidence for diagnostic panels
0
Active trials
10
Pubs (1 yr)
P/LP submissions
P/LP missense
1.37
LOEUF
Mechanism
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GeneReview available — DPH1
Authoritative clinical overview · Recommended first read
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Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.37LOEUF
pLI 0.000
Z-score 0.08
OE 0.98 (0.711.37)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
-0.18Z-score
OE missense 1.03 (0.931.14)
278 obs / 269.8 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?0.98 (0.711.37)
00.351.4
Missense OE?1.03 (0.931.14)
00.61.4
Synonymous OE?1.00
01.21.6
LoF obs/exp: 25 / 25.4Missense obs/exp: 278 / 269.8Syn Z: -0.02

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

DPH1 · protein map & ClinVar variants

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

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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