SP110

Chr 2

SP110 nuclear body protein

Also known as: IFI41, IFI75, IPR1, VODI

The nuclear body is a multiprotein complex that may have a role in the regulation of gene transcription. This gene is a member of the SP100/SP140 family of nuclear body proteins and encodes a leukocyte-specific nuclear body component. The protein can function as an activator of gene transcription and may serve as a nuclear hormone receptor coactivator. In addition, it has been suggested that the protein may play a role in ribosome biogenesis and in the induction of myeloid cell differentiation. Alternative splicing has been observed for this gene and three transcript variants, encoding distinct isoforms, have been identified. [provided by RefSeq, Jul 2008]

GeneReviewsResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtHepatic venoocclusive disease with immunodeficiency

Clinical highlights

Gene-disease validity (ClinGen)
hepatic veno-occlusive disease-immunodeficiency syndrome · ARDefinitivesufficient evidence for diagnostic panels
1
Active trials
11
Pubs (1 yr)
P/LP submissions
P/LP missense
0.94
LOEUF
DN
Mechanism· predicted
📖
GeneReview available — SP110
Authoritative clinical overview · Recommended first read
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Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
0.94LOEUF
pLI 0.000
Z-score 1.83
OE 0.69 (0.510.94)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
0.33Z-score
OE missense 0.95 (0.871.04)
355 obs / 373.2 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.69 (0.510.94)
00.351.4
Missense OE?0.95 (0.871.04)
00.61.4
Synonymous OE?1.01
01.21.6
LoF obs/exp: 28 / 40.6Missense obs/exp: 355 / 373.2Syn Z: -0.12

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

SP110 · protein map & ClinVar variants

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