This gene encodes a mitochondrial matrix protein that belongs to the Lon family of ATP-dependent proteases. This protein mediates the selective degradation of misfolded, unassembled or oxidatively damaged polypeptides in the mitochondrial matrix. It may also have a chaperone function in the assembly of inner membrane protein complexes, and participate in the regulation of mitochondrial gene expression and maintenance of the integrity of the mitochondrial genome. Decreased expression of this gene has been noted in a patient with hereditary spastic paraplegia (PMID:18378094). Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Feb 2013]
Transcription factors with Perturb-seq knockdown data for LONP1. 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 = LONP1 upregulated upon KD; negative = downregulated). The Outlier column indicates whether this gene is in the top or bottom 5% of all TF knockdown effects.
| TF | Mean coef | Binding | Outlier | TF→Gene link |
|---|
Open chromatin peaks (ATAC-seq) in the genomic neighbourhood of LONP1, 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 |
|---|---|---|---|---|
| chr19:5,436,304–5,437,277 | 283.4 kb | Distal (>10kb) Multiome | 227 | |
| chr19:5,536,326–5,536,869 | 183.5 kb | Distal (>10kb) Multiome | 306 | |
| chr19:5,567,504–5,568,201 | 152.3 kb | Distal (>10kb) Multiome | 209 | |
| chr19:5,585,656–5,586,338 | 134.1 kb | Distal (>10kb) Multiome | 267 | |
| chr19:5,621,964–5,623,739 | 97.2 kb | Distal (>10kb) Multiome | 857 | |
| chr19:5,679,757–5,681,464 | 39.3 kb | Distal (>10kb) Multiome | 884 | |
| chr19:5,686,329–5,687,994 | 33.1 kb | Distal (>10kb) Multiome | 693 | |
| chr19:5,689,735–5,690,874 | 29.9 kb | Distal (>10kb) Multiome | 960 | |
| chr19:5,719,717–5,721,174 | 239 bp | At TSS Multiome | 985 | |
| chr19:5,790,527–5,791,509 | 70.8 kb | Distal (>10kb) Multiome | 965 | |
| chr19:5,803,750–5,806,128 | 85.0 kb | Distal (>10kb) Multiome | 858 | |
| chr19:5,826,528–5,829,726 | 107.9 kb | Distal (>10kb) Multiome | 1037 | |
| chr19:5,903,474–5,905,358 | 184.5 kb | Distal (>10kb) Multiome | 1027 | |
| chr19:5,977,406–5,978,787 | 258.1 kb | Distal (>10kb) Multiome | 1028 |
Genomic view of the LONP1 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.