DDX27

Chr 20

DEAD-box helicase 27

Also known as: DRS1, Drs1p, HSPC259, PP3241, RHLP, dJ686N3.1

DEAD box proteins, characterized by the conserved motif Asp-Glu-Ala-Asp (DEAD), are putative RNA helicases. They are implicated in a number of cellular processes involving alteration of RNA secondary structure such as translation initiation, nuclear and mitochondrial splicing, and ribosome and spliceosome assembly. Based on their distribution patterns, some members of this family are believed to be involved in embryogenesis, spermatogenesis, and cellular growth and division. This gene encodes a DEAD box protein involved in the processing of 5.8S and 28S ribosomal RNAs. More specifically, the encoded protein localizes to the nucleolus, where it interacts with the PeBoW complex to ensure proper 3' end formation of 47S rRNA. [provided by RefSeq, Jan 2017]

0
Active trials
7
Pubs (1 yr)
0
P/LP submissions
P/LP missense
0.36
LOEUF
DN
Mechanism· predicted
Clinical SummaryDDX27
Population Constraint (gnomAD)
Moderately constrained gene (pLI 0.58) — some intolerance to loss-of-function variants.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.36LOEUF
pLI 0.583
Z-score 4.96
OE 0.21 (0.130.36)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
1.73Z-score
OE missense 0.77 (0.710.84)
364 obs / 469.7 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.21 (0.130.36)
00.351.4
Missense OE0.77 (0.710.84)
00.61.4
Synonymous OE0.99
01.21.6
LoF obs/exp: 10 / 46.5Missense obs/exp: 364 / 469.7Syn Z: 0.10
DN
0.7326th %ile
GOF
0.5562th %ile
LOF
0.4233th %ile

The highest-scoring mechanism for this gene is dominant-negative.

DNprediction above median

Note: In-silico variant effect predictors (SIFT, PolyPhen, REVEL, CADD) may underestimate pathogenicity of missense variants in genes with GOF or DN mechanisms. Consider functional evidence and clinical context.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

DDX27 · 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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Clinical Literature
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