RFXAP

Chr 13AR

regulatory factor X associated protein

Also known as: MHC2D4

Major histocompatibility (MHC) class II molecules are transmembrane proteins that have a central role in development and control of the immune system. The protein encoded by this gene, along with regulatory factor X-associated ankyrin-containing protein and regulatory factor-5, forms a complex that binds to the X box motif of certain MHC class II gene promoters and activates their transcription. Once bound to the promoter, this complex associates with the non-DNA-binding factor MHC class II transactivator, which controls the cell type specificity and inducibility of MHC class II gene expression. Mutations in this gene have been linked to bare lymphocyte syndrome type II, complementation group D. Transcript variants utilizing different polyA signals have been found for this gene. [provided by RefSeq, Jul 2008]

OMIMResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

MHC class II deficiency 4MIM #620817
AR

Clinical highlights

Gene-disease validity (ClinGen)
MHC class II deficiency · ARDefinitivesufficient evidence for diagnostic panels
0
Active trials
5
Pubs (1 yr)
P/LP submissions
P/LP missense
0.98
LOEUF
Mechanism

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
0.98LOEUF
pLI 0.023
Z-score 1.62
OE 0.43 (0.210.98)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
1.04Z-score
OE missense 0.75 (0.630.88)
99 obs / 132.6 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.43 (0.210.98)
00.351.4
Missense OE?0.75 (0.630.88)
00.61.4
Synonymous OE?0.97
01.21.6
LoF obs/exp: 4 / 9.4Missense obs/exp: 99 / 132.6Syn Z: 0.19

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

RFXAP · protein map & ClinVar variants

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

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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