HID1

Chr 17AR

HID1 domain containing

Also known as: 17orf28, C17orf28, DEE105, DMC1, HID-1

The protein is predicted to function in insulin processing, secretory granule maturation, and vesicle trafficking within the Golgi apparatus and cytosol. Mutations cause developmental and epileptic encephalopathy 105, which typically presents in early childhood with seizures and developmental delays. This gene shows significant constraint against loss-of-function variants (LOEUF 0.47), suggesting that complete loss of protein function is likely pathogenic.

OMIMResearchSummary from RefSeq, UniProt
GOFmechanismARLOEUF 0.471 OMIM phenotype
Clinical SummaryHID1
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.28) despite low pLI — interpret in context.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.47LOEUF
pLI 0.027
Z-score 4.02
OE 0.28 (0.170.47)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
2.27Z-score
OE missense 0.70 (0.640.77)
325 obs / 462.0 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios
LoF OE0.28 (0.170.47)
00.351.4
Missense OE0.70 (0.640.77)
00.61.4
Synonymous OE0.94
01.21.6
LoF obs/exp: 10 / 36.0Missense obs/exp: 325 / 462.0Syn Z: 0.63
DN
0.5672th %ile
GOF
0.73top 25%
LOF
0.3746th %ile

The highest-scoring mechanism for this gene is gain-of-function.

GOFprediction 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

HID1 · 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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Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC