(Q36505469)

14 August 2017

1 reference

title

Diagnosis, pathogenesis, and treatment prospects in cystic kidney disease (English)

1 reference

14 August 2017

Jan Senderek

11

0 references

author name string

Carsten Bergmann

1

1 reference

14 August 2017

Valeska Frank

2

1 reference

14 August 2017

Fabian Küpper

3

1 reference

14 August 2017

Dirk Kamitz

4

1 reference

14 August 2017

Jens Hanten

5

1 reference

14 August 2017

Peter Berges

6

1 reference

14 August 2017

Silke Mager

7

1 reference

14 August 2017

Markus Moser

8

1 reference

14 August 2017

Jutta Kirfel

9

1 reference

14 August 2017

Reinhard Büttner

10

1 reference

14 August 2017

Klaus Zerres

12

1 reference

14 August 2017

publication date

1 January 2006

1 reference

Molecular Diagnosis and Therapy

14 August 2017

published in

1 reference

14 August 2017

volume

10

1 reference

14 August 2017

issue

3

1 reference

14 August 2017

page(s)

163-174

1 reference

https://scigraph.springernature.com/pub.10.1007/bf03256455

14 August 2017

exact match

0 references

cites work

Molecular pathogenesis of ADPKD: the polycystin complex gets complex

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Genetics and pathogenesis of polycystic kidney disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Bilineal disease and trans-heterozygotes in autosomal dominant polycystic kidney disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Polycystic kidney disease: The complete structure of the PKD1 gene and its protein

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

The polycystic kidney disease 1 (PKD1) gene encodes a novel protein with multiple cell recognition domains

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

PKD2, a gene for polycystic kidney disease that encodes an integral membrane protein

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Comparison of phenotypes of polycystic kidney disease types 1 and 2. European PKD1-PKD2 Study Group

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Genotype-renal function correlation in type 2 autosomal dominant polycystic kidney disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Follow-up of intracranial aneurysms in autosomal-dominant polycystic kidney disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Progressive loss of renal function is an age-dependent heritable trait in type 1 autosomal dominant polycystic kidney disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Comparison between siblings and twins supports a role for modifier genes in ADPKD.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Mutation analysis of the entire PKD1 gene: genetic and diagnostic implications

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Location of mutations within the PKD2 gene influences clinical outcome.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Association of mutation position in polycystic kidney disease 1 (PKD1) gene and development of a vascular phenotype.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Perinatal lethality with kidney and pancreas defects in mice with a targetted Pkd1 mutation

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

A human PKD1 transgene generates functional polycystin-1 in mice and is associated with a cystic phenotype

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Cardiovascular, skeletal, and renal defects in mice with a targeted disruption of the Pkd1 gene

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Trans-heterozygous Pkd1 and Pkd2 mutations modify expression of polycystic kidney disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Polycystin 1 is required for the structural integrity of blood vessels

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Cilia and centrosomes: a unifying pathogenic concept for cystic kidney disease?

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Trafficking of TRPP2 by PACS proteins represents a novel mechanism of ion channel regulation

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

The polycystic kidney disease protein PKD2 interacts with Hax-1, a protein associated with the actin cytoskeleton

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Alpha-actinin associates with polycystin-2 and regulates its channel activity

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Polycystin 2 interacts with type I inositol 1,4,5-trisphosphate receptor to modulate intracellular Ca2+ signaling

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Fibrocystin interacts with CAML, a protein involved in Ca2+ signaling

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

The nanomechanics of polycystin-1 extracellular region.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Cleavage of polycystin-1 requires the receptor for egg jelly domain and is disrupted by human autosomal-dominant polycystic kidney disease 1-associated mutations.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

PKD1 interacts with PKD2 through a probable coiled-coil domain

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Co-assembly of polycystin-1 and -2 produces unique cation-permeable currents

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

A novel gene encoding an SH3 domain protein is mutated in nephronophthisis type 1

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Cloning of inv, a gene that controls left/right asymmetry and kidney development.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Inversin, a novel gene in the vertebrate left-right axis pathway, is partially deleted in the inv mouse

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Mutations in INVS encoding inversin cause nephronophthisis type 2, linking renal cystic disease to the function of primary cilia and left-right axis determination

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Mutations in a novel gene, NPHP3, cause adolescent nephronophthisis, tapeto-retinal degeneration and hepatic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

The gene mutated in juvenile nephronophthisis type 4 encodes a novel protein that interacts with nephrocystin

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

A gene mutated in nephronophthisis and retinitis pigmentosa encodes a novel protein, nephroretinin, conserved in evolution

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Characterization of the nephrocystin/nephrocystin-4 complex and subcellular localization of nephrocystin-4 to primary cilia and centrosomes

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Nephrocystin-5, a ciliary IQ domain protein, is mutated in Senior-Loken syndrome and interacts with RPGR and calmodulin

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Positional cloning of a novel gene on chromosome 16q causing Bardet-Biedl syndrome (BBS2)

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Identification of a novel Bardet-Biedl syndrome protein, BBS7, that shares structural features with BBS1 and BBS2.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Identification of the gene for oral-facial-digital type I syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

OFD1 is a centrosomal/basal body protein expressed during mesenchymal-epithelial transition in human nephrogenesis.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Autosomal Recessive Polycystic Kidney Disease: The Clinical Experience in North America

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

The gene mutated in autosomal recessive polycystic kidney disease encodes a large, receptor-like protein

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

PKHD1, the polycystic kidney and hepatic disease 1 gene, encodes a novel large protein containing multiple immunoglobulin-like plexin-transcription-factor domains and parallel beta-helix 1 repeats

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Cellular and subcellular localization of the ARPKD protein; fibrocystin is expressed on primary cilia.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Defects in cholangiocyte fibrocystin expression and ciliary structure in the PCK rat.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

PKHD1 protein encoded by the gene for autosomal recessive polycystic kidney disease associates with basal bodies and primary cilia in renal epithelial cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

The autosomal recessive polycystic kidney disease protein is localized to primary cilia, with concentration in the basal body area

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Polyductin, the PKHD1 gene product, comprises isoforms expressed in plasma membrane, primary cilium, and cytoplasm

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Spectrum of mutations in the gene for autosomal recessive polycystic kidney disease (ARPKD/PKHD1).

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

PKHD1 mutations in autosomal recessive polycystic kidney disease (ARPKD).

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

New options for prenatal diagnosis in autosomal recessive polycystic kidney disease by mutation analysis of the PKHD1 gene

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

PKHD1 mutations in families requesting prenatal diagnosis for autosomal recessive polycystic kidney disease (ARPKD).

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Algorithm for efficient PKHD1 mutation screening in autosomal recessive polycystic kidney disease (ARPKD).

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Comprehensive genomic analysis of PKHD1 mutations in ARPKD cohorts

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Multi-exon deletions of the PKHD1 gene cause autosomal recessive polycystic kidney disease (ARPKD).

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

The polycystic kidney disease proteins, polycystin-1, polycystin-2, polaris, and cystin, are co-localized in renal cilia

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Identification of the gene that, when mutated, causes the human obesity syndrome BBS4.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Mutations in a member of the Ras superfamily of small GTP-binding proteins causes Bardet-Biedl syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Mutations in MKKS cause obesity, retinal dystrophy and renal malformations associated with Bardet-Biedl syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

The Bardet-Biedl protein BBS4 targets cargo to the pericentriolar region and is required for microtubule anchoring and cell cycle progression

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Comparative genomics identifies a flagellar and basal body proteome that includes the BBS5 human disease gene

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Cystic kidney diseases: all roads lead to the cilium

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Polycystins 1 and 2 mediate mechanosensation in the primary cilium of kidney cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Establishing a connection between cilia and Bardet-Biedl Syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

The centrosome in human genetic disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Mechanoregulation of intracellular Ca2+ concentration is attenuated in collecting duct of monocilium-impaired orpk mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Bending the MDCK cell primary cilium increases intracellular calcium

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

The renal cell primary cilium functions as a flow sensor

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Intraflagellar transport and cilia-dependent diseases.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Kidney-specific inactivation of the KIF3A subunit of kinesin-II inhibits renal ciliogenesis and produces polycystic kidney disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

An incredible decade for the primary cilium: a look at a once-forgotten organelle

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Cyclic AMP promotes growth and secretion in human polycystic kidney epithelial cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Calcium restriction allows cAMP activation of the B-Raf/ERK pathway, switching cells to a cAMP-dependent growth-stimulated phenotype

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

The Raf/MEK/ERK pathway: new concepts of activation.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

In vivo effects of activated H-ras oncogene expressed in the liver and in urogenital tissues.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Polycystin-1 activation of c-Jun N-terminal kinase and AP-1 is mediated by heterotrimeric G proteins

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

The polycystic kidney disease 1 gene product modulates Wnt signaling

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Inversin, the gene product mutated in nephronophthisis type II, functions as a molecular switch between Wnt signaling pathways

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Disruption of Bardet-Biedl syndrome ciliary proteins perturbs planar cell polarity in vertebrates

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

PKD1 induces p21(waf1) and regulation of the cell cycle via direct activation of the JAK-STAT signaling pathway in a process requiring PKD2

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Mechanical stimuli induce cleavage and nuclear translocation of the polycystin-1 C terminus

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Polycystin-1, STAT6, and P100 function in a pathway that transduces ciliary mechanosensation and is activated in polycystic kidney disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Emerging therapies for polycystic kidney disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Effectiveness of vasopressin V2 receptor antagonists OPC-31260 and OPC-41061 on polycystic kidney disease development in the PCK rat.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Lillian Jean Kaplan International Prize for advancement in the understanding of polycystic kidney disease. Understanding polycystic kidney disease: a systems biology approach

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

The effect of paclitaxel on the progression of polycystic kidney disease in rodents.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

c-myc as an inducer of polycystic kidney disease in transgenic mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Elevated c-myc protooncogene expression in autosomal recessive polycystic kidney disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

New insights into ADPKD molecular pathways using combination of SAGE and microarray technologies

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

c-myc antisense oligonucleotide treatment ameliorates murine ARPKD.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

EGF receptor tyrosine kinase inhibition attenuates the development of PKD in Han:SPRD rats

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Epidermal growth factor ameliorates autosomal recessive polycystic kidney disease in mice.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Epidermal growth factor receptor tyrosine kinase inhibition is not protective in PCK rats

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Multiorgan mRNA misexpression in murine autosomal recessive polycystic kidney disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Epidermal growth factor receptor expression is abnormal in murine polycystic kidney

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Biliary epithelial cells from mice with congenital polycystic kidney disease are hyperresponsive to epidermal growth factor

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Defective epidermal growth factor gene expression in mice with polycystic kidney disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Rapamycin markedly slows disease progression in a rat model of polycystic kidney disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Caspase inhibition reduces tubular apoptosis and proliferation and slows disease progression in polycystic kidney disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Methylprednisolone retards the progression of inherited polycystic kidney disease in rodents.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Safety and efficacy of long-acting somatostatin treatment in autosomal-dominant polycystic kidney disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Efficacy of octreotide-LAR in dieting women with abdominal obesity and polycystic ovary syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

The Position of the Polycystic Kidney Disease 1 (PKD1) Gene Mutation Correlates with the Severity of Renal Disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Clinical consequences of PKHD1 mutations in 164 patients with autosomal-recessive polycystic kidney disease (ARPKD)

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

A mouse model for cystic biliary dysgenesis in autosomal recessive polycystic kidney disease (ARPKD)

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Milder Presentation of Recessive Polycystic Kidney Disease Requires Presence of Amino Acid Substitution Mutations

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Taxol inhibits progression of congenital polycystic kidney disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF03256455

Disappearance of polycystic kidney disease in revertant c-myc transgenic mice

21 January 2018

1 reference

12 December 2020

Aquaretic effect of a potent, orally active, nonpeptide V2 antagonist in men

1 reference

12 December 2020

A functional floxed allele of Pkd1 that can be conditionally inactivated in vivo

1 reference

12 December 2020

Functional activity of epidermal growth factor receptors in autosomal recessive polycystic kidney disease

1 reference

12 December 2020

Localization of overexpressed c-myc mRNA in polycystic kidneys of the cpk mouse

1 reference

12 December 2020

Effective treatment of an orthologous model of autosomal dominant polycystic kidney disease

1 reference

12 December 2020

Polycystic kidney disease

1 reference

12 December 2020

Increased activity of activator protein-1 transcription factor components ATF2, c-Jun, and c-Fos in human and mouse autosomal dominant polycystic kidney disease

1 reference

12 December 2020

Renal dysfunction but not cystic change is ameliorated by neonatal epidermal growth factor in bpk mice

1 reference

12 December 2020

Somatic inactivation of Pkd2 results in polycystic kidney disease

1 reference

12 December 2020

Management of cerebral aneurysms in autosomal dominant polycystic kidney disease

1 reference

12 December 2020

Treatment of polycystic kidney disease with a novel tyrosine kinase inhibitor

1 reference

12 December 2020

Ciliary signaling goes down the tubes

1 reference

12 December 2020

A complete mutation screen of PKHD1 in autosomal-recessive polycystic kidney disease (ARPKD) pedigrees

1 reference

12 December 2020

Comparison of Pkd1-targeted mutants reveals that loss of polycystin-1 causes cystogenesis and bone defects

1 reference

12 December 2020

A complete mutation screen of the ADPKD genes by DHPLC

1 reference

12 December 2020

Analysis of missense variants in the PKHD1-gene in patients with autosomal recessive polycystic kidney disease (ARPKD).

1 reference

12 December 2020

A novel inhibitor of tumor necrosis factor-alpha converting enzyme ameliorates polycystic kidney disease

1 reference

12 December 2020

Identification of the gene (BBS1) most commonly involved in Bardet-Biedl syndrome, a complex human obesity syndrome

1 reference

12 December 2020

Efficacy of taxol in the orpk mouse model of polycystic kidney disease

1 reference

12 December 2020

Abnormal expression of epidermal growth factor and sulfated glycoprotein SGP-2 messenger RNA in a rat model of autosomal dominant polycystic kidney disease

1 reference

12 December 2020

Linking cilia to Wnts

1 reference

12 December 2020

Inhibition of renal cystic disease development and progression by a vasopressin V2 receptor antagonist

1 reference

12 December 2020

Na-K ATPase activity in murine glucocorticoid induced polycystic kidney disease in vivo.

1 reference

12 December 2020

Autosomal recessive polycystic kidney disease (ARPKD)

1 reference

12 December 2020

Targeting of the rasT24 oncogene to the proximal convoluted tubules in transgenic mice results in hyperplasia and polycystic kidneys

1 reference

12 December 2020

Therapies to slow polycystic kidney disease

1 reference

12 December 2020

Lowering of Pkd1 expression is sufficient to cause polycystic kidney disease

1 reference

12 December 2020

Polycystic kidney disease--the ciliary connection

1 reference

12 December 2020

Renal expression of a transforming growth factor-alpha transgene accelerates the progression of inherited, slowly progressive polycystic kidney disease in the mouse

1 reference

12 December 2020

Excretion of epidermal growth factor in human adult polycystic kidney disease

1 reference

12 December 2020

Identifiers

DOI

10.1007/BF03256455

1 reference

14 August 2017

1 reference