HRG

Chr 3AD

histidine rich glycoprotein

Also known as: HPRG, HRGP, THPH11

This histidine-rich glycoprotein contains two cystatin-like domains and is located in plasma and platelets. The physiological function has not been determined but it is known that the protein binds heme, dyes and divalent metal ions. The encoded protein also has a peptide that displays antimicrobial activity against C. albicans, E. coli, S. aureus, P. aeruginosa, and E. faecalis. It can inhibit rosette formation and interacts with heparin, thrombospondin and plasminogen. Two of the protein's effects, the inhibition of fibrinolysis and the reduction of inhibition of coagulation, indicate a potential prothrombotic effect. Mutations in this gene lead to thrombophilia due to abnormal histidine-rich glycoprotein levels. [provided by RefSeq, Nov 2014]

OMIMResearchGenerating clinical summary…
MultiplemechanismADLOEUF 1.501 OMIM phenotype
Clinical SummaryHRG
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Gene-Disease Validity (ClinGen)
hereditary thrombophilia due to congenital histidine-rich (poly-L) glycoprotein deficiency · ADModerate

Moderate evidence — consider for supplementary testing

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
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Clinical Trials
5 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?
1.50LOEUF
pLI 0.000
Z-score -0.12
OE 1.03 (0.721.50)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
-0.37Z-score
OE missense 1.06 (0.971.17)
308 obs / 290.1 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?1.03 (0.721.50)
00.351.4
Missense OE?1.06 (0.971.17)
00.61.4
Synonymous OE?1.00
01.21.6
LoF obs/exp: 20 / 19.4Missense obs/exp: 308 / 290.1Syn Z: -0.01

This gene — mechanism propensity

DN
0.6841th %ile
GOF
0.4184th %ile
LOF
0.3940th %ile

This gene has evidence for multiple mechanisms of pathogenicity (dominant-negative and loss-of-function). Both the Badonyi & Marsh prediction and the broader genomic evidence point to dominant-negative as the predominant mechanism. Different variants in this gene may act through different mechanisms — interpret in context of the specific variant.

DNprediction above median
LOF1 literature citation

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.

Literature Evidence

LOFQuantity analysis of plasma HRG revealed approximately half concentration in affected individuals compared to that in unaffected controls, suggesting haploinsufficiency as the pathogenic mechanism.1

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

References

  1. 1.PMID 29108964

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

HRG · protein map & ClinVar variants

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