HABP4

Chr 9

hyaluronan binding protein 4

Also known as: IHABP-4, IHABP4, Ki-1/57, SERBP1L

The protein encoded by this gene is a ribosome-binding protein that promotes ribosome hibernation and stabilization, and also regulates RNA splicing and transcriptional processes. Mutations cause autosomal recessive spastic paraplegia with intellectual disability, typically presenting in early childhood with progressive spasticity primarily affecting the lower extremities and developmental delays. The gene shows moderate constraint against loss-of-function variants, suggesting some intolerance to complete protein loss.

OMIMResearchSummary from RefSeq, UniProt
DNmechanismLOEUF 0.70
Clinical SummaryHABP4
Population Constraint (gnomAD)
Low constraint (pLI 0.01) — loss-of-function variants are relatively tolerated in the population.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.70LOEUF
pLI 0.006
Z-score 2.53
OE 0.37 (0.210.70)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
1.13Z-score
OE missense 0.78 (0.690.89)
168 obs / 214.4 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.37 (0.210.70)
00.351.4
Missense OE0.78 (0.690.89)
00.61.4
Synonymous OE0.81
01.21.6
LoF obs/exp: 7 / 18.9Missense obs/exp: 168 / 214.4Syn Z: 1.30
DN
0.6648th %ile
GOF
0.5268th %ile
LOF
0.4529th %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

HABP4 · 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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