HOXD8

Chr 2

homeobox D8

Also known as: HOX4, HOX4E, HOX5.4

This gene belongs to the homeobox family of genes. The homeobox genes encode a highly conserved family of transcription factors that play an important role in morphogenesis in all multicellular organisms. Mammals possess four similar homeobox gene clusters, HOXA, HOXB, HOXC and HOXD, located on different chromosomes, consisting of 9 to 11 genes arranged in tandem. This gene is one of several homeobox HOXD genes located in a cluster on chromosome 2. Deletions that remove the entire HOXD gene cluster or the 5' end of this cluster have been associated with severe limb and genital abnormalities. In addition to effects during embryogenesis, this particular gene may also play a role in adult urogenital tract function. Alternate splicing results in multiple transcript variants.[provided by RefSeq, Dec 2010]

OMIMResearchGenerating clinical summary…
DNmechanismLOEUF 0.85
Clinical SummaryHOXD8
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.33) despite low pLI — interpret in context.
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ClinVar Variants
64 VUS of 71 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
0.85LOEUF
pLI 0.105
Z-score 1.88
OE 0.33 (0.150.85)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
-1.42Z-score
OE missense 1.34 (1.191.52)
182 obs / 135.6 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?0.33 (0.150.85)
00.351.4
Missense OE?1.34 (1.191.52)
00.61.4
Synonymous OE?1.38
01.21.6
LoF obs/exp: 3 / 9.1Missense obs/exp: 182 / 135.6Syn Z: -2.28

This gene — mechanism propensity

DN
0.6840th %ile
GOF
0.4283th %ile
LOF
0.60top 25%

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

71 submitted variants in ClinVar

Classification Summary

VUS64
Likely Benign1
64
VUS
1
Likely Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
0
0
0
Likely Pathogenic
0
0
0
0
0
VUS
0
64
0
0
64
Likely Benign
0
0
0
1
1
Benign
0
0
0
0
0
Total0640165

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

29 pathogenic / likely-pathogenic (of 31) ClinVar copy-number / structural variants overlap HOXD8 — these span large chromosomal regions, not the gene specifically, and are excluded from the counts above. Explore in CNV tools →

Protein Context — Lollipop Plot

HOXD8 · 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.

Search ClinicalTrials.gov →