IKZF5

Chr 10AD

IKAROS family zinc finger 5

Also known as: PEGASUS, THC7, ZNFN1A5

Members of the Ikaros (ZNFN1A1; MIM 603023) family of transcription factors, which includes Pegasus, are expressed in lymphocytes and are implicated in the control of lymphoid development.[supplied by OMIM, Jul 2002]

OMIMResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

Thrombocytopenia, autosomal dominant, 7MIM #619130
AD
UniProtThrombocytopenia 7

Clinical highlights

Gene-disease validity (ClinGen)
thrombocytopenia 7 · ADModerateconsider for supplementary testing
Interpreting a novel variant
This gene is strongly intolerant of loss-of-function variation in the population, so LoF variants warrant close attention. No curated mechanism annotation is available — see the mechanism card for the computational prediction and its caveats.Based on population constraint only.
1
Active trials
Pubs (1 yr)
P/LP submissions
P/LP missense
0.46
LOEUF
LOF
Mechanism· predicted

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint?
0.46LOEUF
pLI 0.780
Z-score 2.94
OE 0.15 (0.060.46)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint?
1.89Z-score
OE missense 0.65 (0.570.75)
155 obs / 236.8 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.15 (0.060.46)
00.351.4
Missense OE?0.65 (0.570.75)
00.61.4
Synonymous OE?0.88
01.21.6
LoF obs/exp: 2 / 13.8Missense obs/exp: 155 / 236.8Syn Z: 0.87

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

IKZF5 · protein map & ClinVar variants

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