PHF8

Chr XXLR

PHD finger protein 8

Also known as: JHDM1F, KDM7B, MRXSSD, ZNF422

The protein functions as a histone lysine demethylase that removes methyl groups from histones to activate gene transcription, requiring Fe(2+), 2-oxoglutarate, and oxygen for catalytic activity. Loss-of-function mutations cause X-linked syndromic intellectual disability of the Siderius type, which is inherited in an X-linked recessive pattern. The pathogenic mechanism involves disrupted chromatin remodeling and transcriptional regulation due to impaired histone demethylase activity.

OMIMResearchSummary from RefSeq, OMIM, UniProt, Mechanism
LOFmechanismXLRLOEUF 0.261 OMIM phenotype
Clinical SummaryPHF8
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Gene-Disease Validity (ClinGen)
syndromic X-linked intellectual disability Siderius type · XLDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 1.00). One damaged copy is likely sufficient to cause disease.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Dual constrained — LoF & missense intolerant
LoF Constraint
0.26LOEUF
pLI 0.998
Z-score 5.13
OE 0.12 (0.060.26)
Highly constrained

Highly LoF-intolerant (top ~10% of genes)

Missense Constraint
3.98Z-score
OE missense 0.46 (0.410.52)
199 obs / 431.4 exp
Constrained

Highly missense-constrained (top ~0.1%)

Observed / Expected Ratios
LoF OE0.12 (0.060.26)
00.351.4
Missense OE0.46 (0.410.52)
00.61.4
Synonymous OE0.96
01.21.6
LoF obs/exp: 5 / 40.0Missense obs/exp: 199 / 431.4Syn Z: 0.43
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitivePHF8-related syndromic intellectual developmental disorder, Siderius typeLOFXLR
DN
0.2898th %ile
GOF
0.2895th %ile
LOF
0.82top 5%

The highest-scoring mechanism for this gene is loss-of-function (haploinsufficiency).

LOFprediction above median · LOEUF 0.26

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312. Mechanism ranking also informed by gnomAD constraint, ClinVar, and ClinGen data.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

PHF8 · protein map & ClinVar variants

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

3D Protein StructureAlphaFold

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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Clinical Literature
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