Article

The C-terminal tail of polycystin-1 suppresses cystic disease in a mitochondrial enzyme-dependent fashion

2021-12-23

Abstract excerpt

<h4>SUMMARY</h4> Autosomal dominant polycystic kidney disease (ADPKD) is the most prevalent potentially lethal monogenic disorder. Approximately 78% of cases are caused by mutations in the PKD1 gene, which encodes polycystin-1 (PC1). PC1 is a large 462-kDa protein that undergoes cleavage in its N and C-terminal domains. C-terminal cleavage produces fragments that translocate to mitochondria. We show that transge...

Topics

Open a Topic to create a Post that cites this publication.

Identifiers and source

Literature Corpus work: e311dcfb-4f01-516b-94e1-125568551d10
DOI: 10.1101/2021.12.21.473680

Open publication

Related research

Semantic proximity does not establish scientific evidence.

Click a neighbor to travelStep 1 · 12 closest

The C-terminal tail of polycystin-1 suppresses cystic disease in a mitochondrial enzyme-dependent fashionDOI 10.1101/2021.12.21.473680Read

The C-terminal tail of polycystin-1 suppresses cystic disease in a mitochondrial enzyme-dependent fashionSemantic proximity Read

Use of a novel knock-in allele of <i>Pkd1</i> identifies nicotinamide nucleotide dehydrogenase as a mitochondrial binding partner of polycystin-1Semantic proximity Read

Fibrocystin/Polyductin releases a C-terminal fragment that translocates into mitochondria and prevents cystogenesisSemantic proximity Read

A cleavage product of Polycystin-1 is a mitochondrial matrix protein that affects mitochondria morphology and function when heterologously expressedSemantic proximity Read

A polycystin-centric view of cyst formation and disease: the polycystins revisited.Semantic proximity Read

Metabolic Derangement in Polycystic Kidney Disease Mouse Models Is Ameliorated by Mitochondrial-Targeted AntioxidantsSemantic proximity Read

Human-Specific Abnormal Alternative Splicing of Wild-Type PKD1 Induces Premature Termination of Polycystin-1.Semantic proximity Read

Polycystin-1 cleavage and the regulation of transcriptional pathways.Semantic proximity Read

Defining a link with autosomal-dominant polycystic kidney disease in mice with congenitally low expression of Pkd1.Semantic proximity Read

Polycystic kidney disease: The complete structure of the PKD1 gene and its proteinSemantic proximity Read

A mouse model for polycystic kidney disease through a somatic in-frame deletion in the 5' end of Pkd1.Semantic proximity Read

Anti-Polyamine Therapy Restrains Kidney Cyst Growth in an Orthologous Mouse Model of Autosomal Dominant Polycystic Kidney DiseaseSemantic proximity Read