PHF10

Chr 6

PHD finger protein 10

Also known as: BAF45A, SMARCG4, XAP135

PHF10 encodes a chromatin remodeling protein that belongs to the neural progenitor-specific BAF complex (npBAF) and regulates transcription by switching from stem cell to neuronal chromatin remodeling during brain development. Mutations cause Bainbridge-Ropers syndrome, an autosomal dominant neurodevelopmental disorder characterized by intellectual disability, developmental delay, autism spectrum disorder, and distinctive facial features. The gene shows tolerance to loss-of-function variants (low pLI score), suggesting the disorder may involve complex mechanisms affecting chromatin remodeling in developing neural tissue.

OMIMResearchSummary from RefSeq, UniProt
DNmechanismLOEUF 0.64
Clinical SummaryPHF10
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.64LOEUF
pLI 0.000
Z-score 3.01
OE 0.39 (0.240.64)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
1.79Z-score
OE missense 0.68 (0.600.78)
173 obs / 252.9 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.39 (0.240.64)
00.351.4
Missense OE0.68 (0.600.78)
00.61.4
Synonymous OE1.02
01.21.6
LoF obs/exp: 11 / 28.3Missense obs/exp: 173 / 252.9Syn Z: -0.16
DN
0.6453th %ile
GOF
0.5170th %ile
LOF
0.4332th %ile

The highest-scoring mechanism for this gene is dominant-negative.

DNprediction above median

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.

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

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

PHF10 · 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
Open Research Assistant →
Key Publications
Landmark & review papers · by relevance
PubMed
Top 3 results · since 2015Search PubMed ↗