HEBP1

Chr 12

heme binding protein 1

Also known as: HBP, HEBP

The protein binds heme and porphyrins with high affinity and may facilitate removal of potentially toxic free porphyrinogens from cells. Mutations cause autosomal recessive intellectual disability with seizures and spasticity, typically presenting in early childhood. This gene is highly constrained against loss-of-function mutations, indicating that complete loss of protein function is likely not tolerated.

Summary from RefSeq, UniProt
Research Assistant →
2
Active trials
6
Pubs (1 yr)
45
P/LP submissions
0%
P/LP missense
1.76
LOEUF
Mechanism
Clinical SummaryHEBP1
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
45 unique Pathogenic / Likely Pathogenic· 27 VUS of 80 total submissions
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Clinical Trials
2 active or recruiting trials — potential therapeutic options may be available

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.76LOEUF
pLI 0.000
Z-score -0.11
OE 1.04 (0.611.76)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.09Z-score
OE missense 0.98 (0.841.14)
115 obs / 117.9 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE1.04 (0.611.76)
00.351.4
Missense OE0.98 (0.841.14)
00.61.4
Synonymous OE1.15
01.21.6
LoF obs/exp: 8 / 7.7Missense obs/exp: 115 / 117.9Syn Z: -0.80

ClinVar Variant Classifications

80 submitted variants in ClinVar

Classification Summary

Pathogenic43
Likely Pathogenic2
VUS27
43
Pathogenic
2
Likely Pathogenic
27
VUS

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
43
0
43
Likely Pathogenic
0
0
2
0
2
VUS
0
22
5
0
27
Likely Benign
0
0
0
0
0
Benign
0
0
0
0
0
Total02250072

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

View in ClinVar →

Protein Context — Lollipop Plot

HEBP1 · protein map & ClinVar variants

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

Clinical Literature
Open Research Assistant →
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC