PKHD1
PKHD1 ciliary IPT domain containing fibrocystin/polyductin | ARPKD, FCYT, FPC, TIGM1

The protein encoded by this gene is predicted to have a single transmembrane (TM)-spanning domain and multiple copies of an immunoglobulin-like plexin-transcription-factor domain. Alternative splicing results in two transcript variants encoding different isoforms. Other alternatively spliced transcripts have been described, but the full length sequences have not been determined. Several of these transcripts are predicted to encode truncated products which lack the TM and may be secreted. Mutations in this gene cause autosomal recessive polycystic kidney disease, also known as polycystic kidney and hepatic disease-1. [provided by RefSeq, Jul 2008]

Biological processes 53 terms
9+0 non-motile cilium (GO:0097731)Golgi apparatus (GO:0005794)Golgi apparatus (GO:0005794)apical plasma membrane (GO:0016324)apical plasma membrane (GO:0016324)branching morphogenesis of an epithelial tube (GO:0048754)branching morphogenesis of an epithelial tube (GO:0048754)cell-cell adhesion (GO:0098609)cell-cell junction organization (GO:0045216)centrosome (GO:0005813)chromosome, centromeric region (GO:0000775)ciliary basal body (GO:0036064)cilium (GO:0005929)cilium (GO:0005929)cilium (GO:0005929)cytoplasm (GO:0005737)cytoplasm (GO:0005737)cytosol (GO:0005829)endoplasmic reticulum (GO:0005783)endoplasmic reticulum (GO:0005783)epithelial cell morphogenesis (GO:0003382)establishment of centrosome localization (GO:0051660)establishment of mitotic spindle orientation (GO:0000132)establishment of mitotic spindle orientation (GO:0000132)external side of plasma membrane (GO:0009897)extracellular exosome (GO:0070062)extracellular exosome (GO:0070062)extracellular exosome (GO:0070062)extracellular region (GO:0005576)homeostatic process (GO:0042592)intracellular calcium ion homeostasis (GO:0006874)mitotic spindle (GO:0072686)negative regulation of apoptotic process (GO:0043066)negative regulation of epithelial cell apoptotic process (GO:1904036)negative regulation of epithelial cell apoptotic process (GO:1904036)nucleus (GO:0005634)perinuclear region of cytoplasm (GO:0048471)plasma membrane (GO:0005886)positive regulation of cell population proliferation (GO:0008284)positive regulation of epithelial cell proliferation (GO:0050679)positive regulation of epithelial cell proliferation (GO:0050679)protein binding (GO:0005515)regulation of ERK1 and ERK2 cascade (GO:0070372)regulation of TOR signaling (GO:0032006)regulation of cell adhesion (GO:0030155)regulation of cell-cell adhesion (GO:0022407)regulation of cell-matrix adhesion (GO:0001952)regulation of centrosome duplication (GO:0010824)regulation of cholangiocyte proliferation (GO:1904054)regulation of establishment of planar polarity (GO:0090175)regulation of establishment of planar polarity (GO:0090175)signaling receptor activity (GO:0038023)spindle (GO:0005819)
Expression (TPM)
PKHD1 — as a Regulated Gene

TFs regulating PKHD1 0 TFs

Transcription factors with Perturb-seq knockdown data for PKHD1. 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 = PKHD1 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 PKHD1

Open chromatin peaks (ATAC-seq) in the genomic neighbourhood of PKHD1, 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
chr6:52,076,807–52,077,726 9.9 kb Proximal (<10kb) 279
chr6:52,087,502–52,087,671 at TSS At TSS 59
chr6:52,087,797–52,087,972 187 bp At TSS 105

Genome Browser

Genomic view of the PKHD1 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.

chr6:52,066,807 – 52,097,972
Proximal 1 kb Distal 10 kb Multiome HiCAR ATAC-seq RNA-seq