TWNK
twinkle mtDNA helicase | FLJ21832, PEO, PEO1, TWINKLE, TWINL, C10orf2, IOSCA

This gene encodes a hexameric DNA helicase which unwinds short stretches of double-stranded DNA in the 5' to 3' direction and, along with mitochondrial single-stranded DNA binding protein and mtDNA polymerase gamma, is thought to play a key role in mtDNA replication. The protein localizes to the mitochondrial matrix and mitochondrial nucleoids. Mutations in this gene cause infantile onset spinocerebellar ataxia (IOSCA) and progressive external ophthalmoplegia (PEO) and are also associated with several mitochondrial depletion syndromes. Alternative splicing results in multiple transcript variants encoding distinct isoforms.[provided by RefSeq, Aug 2009]

Developmental clusters: GC4
Biological processes 30 terms
Expression (TPM)
TWNK — as a Regulated Gene

TFs regulating TWNK 0 TFs

Transcription factors with Perturb-seq knockdown data for TWNK. 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 = TWNK 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 TWNK

Open chromatin peaks (ATAC-seq) in the genomic neighbourhood of TWNK, 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
chr10:100,715,555–100,715,963 271.7 kb Distal (>10kb) Multiome 117
chr10:100,729,074–100,730,178 258.1 kb Distal (>10kb) Multiome 304
chr10:100,744,447–100,746,357 241.9 kb Distal (>10kb) Multiome 379
chr10:100,912,455–100,913,519 74.7 kb Distal (>10kb) Multiome 906
chr10:100,968,950–100,970,567 18.2 kb Distal (>10kb) Multiome 681
chr10:100,987,003–100,987,794 94 bp At TSS Multiome 786
chr10:100,994,403–100,994,731 6.9 kb Proximal (<10kb) 101
chr10:100,996,424–100,998,696 9.4 kb Proximal (<10kb) Multiome 945
chr10:100,998,810–101,000,165 12.0 kb Distal (>10kb) Multiome 845
chr10:101,018,377–101,019,461 31.5 kb Distal (>10kb) Multiome 654
chr10:101,030,588–101,032,521 43.6 kb Distal (>10kb) Multiome 613
chr10:101,042,527–101,043,387 55.4 kb Distal (>10kb) Multiome 655
chr10:101,048,051–101,048,547 60.7 kb Distal (>10kb) Multiome 87
chr10:101,049,966–101,050,753 62.8 kb Distal (>10kb) Multiome 412
chr10:101,060,073–101,063,211 75.2 kb Distal (>10kb) Multiome 1124
chr10:101,066,181–101,066,983 79.0 kb Distal (>10kb) Multiome 734
chr10:101,067,105–101,068,123 80.3 kb Distal (>10kb) Multiome 643
chr10:101,095,368–101,096,269 108.2 kb Distal (>10kb) Multiome 326
chr10:101,131,094–101,132,387 143.8 kb Distal (>10kb) Multiome 289
chr10:101,139,407–101,140,520 152.3 kb Distal (>10kb) Multiome 158
chr10:101,161,678–101,162,270 174.4 kb Distal (>10kb) Multiome 336
chr10:101,214,601–101,215,378 227.4 kb Distal (>10kb) Multiome 302
chr10:101,217,392–101,218,117 230.2 kb Distal (>10kb) Multiome 203
chr10:101,236,527–101,237,332 249.5 kb Distal (>10kb) Multiome 123
chr10:101,285,534–101,286,046 298.3 kb Distal (>10kb) Multiome 150

Genome Browser

Genomic view of the TWNK 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.

chr10:100,705,555 – 101,296,046
Proximal 1 kb Distal 10 kb Multiome HiCAR ATAC-seq RNA-seq