OPHN1
oligophrenin 1 | ARHGAP41, OPN1, MRX60

This gene encodes a Rho-GTPase-activating protein that promotes GTP hydrolysis of Rho subfamily members. Rho proteins are important mediators of intracellular signal transduction, which affects cell migration and cell morphogenesis. Mutations in this gene are responsible for OPHN1-related X-linked cognitive disability with cerebellar hypoplasia and distinctive facial dysmorhphism. [provided by RefSeq, Jul 2008]

Member of: DE-3 DE-3.3
Biological processes 49 terms
GTPase activator activity (GO:0005096)GTPase activator activity (GO:0005096)GTPase activator activity (GO:0005096)actin binding (GO:0003779)actin cytoskeleton (GO:0015629)actin cytoskeleton (GO:0015629)actin cytoskeleton organization (GO:0030036)axon (GO:0030424)axon guidance (GO:0007411)cell junction assembly (GO:0034329)cell morphogenesis involved in neuron differentiation (GO:0048667)cerebellar granule cell differentiation (GO:0021707)cerebral cortex neuron differentiation (GO:0021895)cytoplasm (GO:0005737)cytoplasm (GO:0005737)dendrite (GO:0030425)dendritic spine (GO:0043197)dendritic spine (GO:0043197)establishment of epithelial cell apical/basal polarity (GO:0045198)glutamatergic synapse (GO:0098978)glutamatergic synapse (GO:0098978)glutamatergic synapse (GO:0098978)glutamatergic synapse (GO:0098978)ionotropic glutamate receptor binding (GO:0035255)maintenance of postsynaptic specialization structure (GO:0098880)negative regulation of proteasomal protein catabolic process (GO:1901799)negative regulation of proteasomal protein catabolic process (GO:1901799)nervous system development (GO:0007399)neuron differentiation (GO:0030182)neuron projection development (GO:0031175)phospholipid binding (GO:0005543)postsynapse (GO:0098794)presynapse (GO:0098793)presynapse (GO:0098793)regulation of Rho protein signal transduction (GO:0035023)regulation of endocytosis (GO:0030100)regulation of endocytosis (GO:0030100)regulation of postsynaptic neurotransmitter receptor internalization (GO:0099149)regulation of postsynaptic neurotransmitter receptor internalization (GO:0099149)regulation of postsynaptic neurotransmitter receptor internalization (GO:0099149)regulation of synaptic transmission, glutamatergic (GO:0051966)regulation of synaptic vesicle endocytosis (GO:1900242)signal transduction (GO:0007165)signal transduction (GO:0007165)substrate-dependent cell migration, cell extension (GO:0006930)synapse (GO:0045202)synaptic vesicle endocytosis (GO:0048488)terminal bouton (GO:0043195)terminal bouton (GO:0043195)
Expression (TPM)
OPHN1 — as a Regulated Gene

TFs regulating OPHN1 0 TFs

Transcription factors with Perturb-seq knockdown data for OPHN1. The Binding column indicates whether any binding evidence exists for this TF–gene pair (ChIP-seq or motif footprint peaks). The Mean coef is the average Perturb-seq regression coefficient across active gRNAs (positive = OPHN1 upregulated upon KD; negative = downregulated). The Outlier column indicates whether this gene is in the top or bottom 5% of all TF knockdown effects.

Data: Effect:
TF Mean coef Binding Outlier TF→Gene link

Elements linked to OPHN1

Open chromatin peaks (ATAC-seq) in the genomic neighbourhood of OPHN1, linked by TSS proximity or chromatin conformation (Multiome / HiCAR). Each element overlaps at least one TF ChIP-seq binding site — the TFs column shows how many distinct TFs bind that element.

Accessibility Element Dist. to TSS Link type TFs
chrX:68,432,740–68,434,271 199 bp At TSS Multiome 531
chrX:68,498,399–68,499,332 65.1 kb Distal (>10kb) Multiome 697
chrX:68,686,156–68,686,853 252.6 kb Distal (>10kb) Multiome 235
chrX:68,693,384–68,694,277 259.8 kb Distal (>10kb) Multiome 303

Genome Browser

Genomic view of the OPHN1 locus showing ATAC-seq accessibility and RNA-seq expression across the ESC → DE time course, together with TF binding peaks and element-to-TSS loop connections.

chrX:68,422,740 – 68,704,277
Proximal 1 kb Distal 10 kb Multiome HiCAR ATAC-seq RNA-seq