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Search results 1 to 100 out of 229 for Pkhd1

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0.202s
Type Details Score
Gene
Type: gene
Organism: Homo sapiens
Gene
Type: gene
Organism: Rattus norvegicus
Protein
Organism: Mus musculus
Length: 4060  
Fragment?: false
Protein Domain
Type: Family
Description: Fibrocystin, also known as polyductin or polycystic kidney and hepatic disease 1 protein (PKHD1), may be required for correct bipolar cell division through the regulation of centrosome duplication and mitotic spindle assembly []. There are several alternative forms of fibrocystin which are expressed in plasma membrane, primary cilium, and cytoplasm []. The mature cleaved form of fibrocystin is found in exosome-like vesicles (ELVs) in mouse urine. These fibrocystin-containing ELVs can rapidly associate with primary cilia in the kidney []. Mutations in fibrocystin gene cause polycystic kidney disease, autosomal recessive (ARPKD), which is a severe form of polycystic kidney disease affecting the kidneys and the hepatic biliary tract [].
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus musculus
Publication
First Author: Zhang MZ
Year: 2004
Journal: Proc Natl Acad Sci U S A
Title: PKHD1 protein encoded by the gene for autosomal recessive polycystic kidney disease associates with basal bodies and primary cilia in renal epithelial cells.
Volume: 101
Issue: 8
Pages: 2311-6
Publication
First Author: Williams SS
Year: 2014
Journal: Am J Physiol Renal Physiol
Title: Tissue-specific regulation of the mouse Pkhd1 (ARPKD) gene promoter.
Volume: 307
Issue: 3
Pages: F356-68
Gene
Type: gene
Organism: Homo sapiens
Publication
First Author: Nishio S
Year: 2010
Journal: J Am Soc Nephrol
Title: Loss of oriented cell division does not initiate cyst formation.
Volume: 21
Issue: 2
Pages: 295-302
Publication
First Author: Patel V
Year: 2013
Journal: Proc Natl Acad Sci U S A
Title: miR-17~92 miRNA cluster promotes kidney cyst growth in polycystic kidney disease.
Volume: 110
Issue: 26
Pages: 10765-70
Publication
First Author: Fedeles SV
Year: 2011
Journal: Nat Genet
Title: A genetic interaction network of five genes for human polycystic kidney and liver diseases defines polycystin-1 as the central determinant of cyst formation.
Volume: 43
Issue: 7
Pages: 639-47
Publication
First Author: Bakeberg JL
Year: 2011
Journal: J Am Soc Nephrol
Title: Epitope-tagged Pkhd1 tracks the processing, secretion, and localization of fibrocystin.
Volume: 22
Issue: 12
Pages: 2266-77
Publication
First Author: Menezes LF
Year: 2004
Journal: Kidney Int
Title: Polyductin, the PKHD1 gene product, comprises isoforms expressed in plasma membrane, primary cilium, and cytoplasm.
Volume: 66
Issue: 4
Pages: 1345-55
Publication
First Author: Mai W
Year: 2005
Journal: Mol Biol Cell
Title: Inhibition of Pkhd1 impairs tubulomorphogenesis of cultured IMCD cells.
Volume: 16
Issue: 9
Pages: 4398-409
Publication
First Author: Hu B
Year: 2011
Journal: Exp Cell Res
Title: Cystogenesis in ARPKD results from increased apoptosis in collecting duct epithelial cells of Pkhd1 mutant kidneys.
Volume: 317
Issue: 2
Pages: 173-87
Publication
First Author: Hiesberger T
Year: 2004
Journal: J Clin Invest
Title: Mutation of hepatocyte nuclear factor-1beta inhibits Pkhd1 gene expression and produces renal cysts in mice.
Volume: 113
Issue: 6
Pages: 814-25
Publication
First Author: Hiesberger T
Year: 2005
Journal: J Biol Chem
Title: Role of the hepatocyte nuclear factor-1beta (HNF-1beta) C-terminal domain in Pkhd1 (ARPKD) gene transcription and renal cystogenesis.
Volume: 280
Issue: 11
Pages: 10578-86
Publication
First Author: Richards T
Year: 2019
Journal: Biochim Biophys Acta Mol Basis Dis
Title: Atmin modulates Pkhd1 expression and may mediate Autosomal Recessive Polycystic Kidney Disease (ARPKD) through altered non-canonical Wnt/Planar Cell Polarity (PCP) signalling.
Volume: 1865
Issue: 2
Pages: 378-390
Allele
Name: transgene insertion 1, Peter Igarashi
Allele Type: Transgenic
Attribute String: Recombinase
Publication categories: ProxyCollection
Publication
First Author: Ward CJ
Year: 2002
Journal: Nat Genet
Title: The gene mutated in autosomal recessive polycystic kidney disease encodes a large, receptor-like protein.
Volume: 30
Issue: 3
Pages: 259-69
Protein
Organism: Mus musculus
Length: 4059  
Fragment?: false
Protein
Organism: Mus musculus
Length: 4059  
Fragment?: false
Genotype
Symbol: Pkd2/Pkd2 Tg(Pkhd1-cre)1Igr/?
Background: Not Specified
Zygosity: cn
Has Mutant Allele: true
Genotype
Symbol: Pkd2/Pkd2 Tg(Pkhd1-cre)1Igr/?
Background: involves: 129S4/SvJae * C57BL/6
Zygosity: cn
Has Mutant Allele: true
Genotype
Symbol: Pkd2/Pkd2 Tg(Pkhd1-cre)1Igr/?
Background: Not Specified
Zygosity: cn
Has Mutant Allele: true
Protein
Organism: Mus musculus
Length: 187  
Fragment?: false
Protein
Organism: Mus musculus
Length: 151  
Fragment?: true
Publication
First Author: Zhang J
Year: 2010
Journal: Hum Mol Genet
Title: Polycystic kidney disease protein fibrocystin localizes to the mitotic spindle and regulates spindle bipolarity.
Volume: 19
Issue: 17
Pages: 3306-19
Publication
First Author: Patel V
Year: 2011
Journal: J Am Soc Nephrol
Title: Tagged fibrocystin sheds its secrets.
Volume: 22
Issue: 12
Pages: 2148-50
Genotype
Symbol: Mirc1/Mirc1 Pkd2/Pkd2 Tg(Pkhd1-cre)1Igr/?
Background: involves: 129S4/SvJae * C57BL/6
Zygosity: cn
Has Mutant Allele: true
Publication
First Author: Wang Q
Year: 2018
Journal: Kidney Int
Title: Adenylyl cyclase 5 deficiency reduces renal cyclic AMP and cyst growth in an orthologous mouse model of polycystic kidney disease.
Volume: 93
Issue: 2
Pages: 403-415
Publication
First Author: Zhou X
Year: 2015
Journal: Hum Mol Genet
Title: Therapeutic targeting of BET bromodomain protein, Brd4, delays cyst growth in ADPKD.
Volume: 24
Issue: 14
Pages: 3982-93
Publication
First Author: Cao Y
Year: 2009
Journal: Proc Natl Acad Sci U S A
Title: Chemical modifier screen identifies HDAC inhibitors as suppressors of PKD models.
Volume: 106
Issue: 51
Pages: 21819-24
Genotype
Symbol: Pkd1/Pkd1 Tg(Pkhd1-cre)1Igr/?
Background: involves: C57BL/6 * SJL
Zygosity: cn
Has Mutant Allele: true
Genotype
Symbol: Pkd2/Pkd2 Tg(Pdk1)248Som/? Tg(Pkhd1-cre)1Igr/?
Background: involves: C57BL/6J * SJL/J
Zygosity: cn
Has Mutant Allele: true
Genotype
Symbol: Mir21a/Mir21a Pkd2/Pkd2 Tg(Pkhd1-cre)1Igr/?
Background: involves: 129S/SvEv
Zygosity: cn
Has Mutant Allele: true
Publication
First Author: Huang W
Year: 2018
Journal: J Immunol
Title: A Novel Pkhd1 Mutation Interacts with the Nonobese Diabetic Genetic Background To Cause Autoimmune Cholangitis.
Volume: 200
Issue: 1
Pages: 147-162
Publication
First Author: Aboudehen K
Year: 2018
Journal: J Biol Chem
Title: Long noncoding RNA Hoxb3os is dysregulated in autosomal dominant polycystic kidney disease and regulates mTOR signaling.
Volume: 293
Issue: 24
Pages: 9388-9398
Publication
First Author: Chen L
Year: 2015
Journal: J Clin Invest
Title: Macrophage migration inhibitory factor promotes cyst growth in polycystic kidney disease.
Volume: 125
Issue: 6
Pages: 2399-412
Publication
First Author: Flowers EM
Year: 2018
Journal: Nat Commun
Title: Lkb1 deficiency confers glutamine dependency in polycystic kidney disease.
Volume: 9
Issue: 1
Pages: 814
Publication
First Author: Cai Y
Year: 2014
Journal: J Clin Invest
Title: Altered trafficking and stability of polycystins underlie polycystic kidney disease.
Volume: 124
Issue: 12
Pages: 5129-44
Genotype
Symbol: Gt(ROSA)26Sor/Gt(ROSA)26Sor<+> Kif3a/Kif3a<+> Tg(Pkhd1-cre)1Igr/?
Background: involves: 129S1/Sv * 129S4/SvJaeSor * 129X1/SvJ
Zygosity: cn
Has Mutant Allele: true
Publication  
First Author: Hajarnis S
Year: 2017
Journal: Nat Commun
Title: microRNA-17 family promotes polycystic kidney disease progression through modulation of mitochondrial metabolism.
Volume: 8
Pages: 14395
Genotype
Symbol: Gt(ROSA)26Sor/Gt(ROSA)26Sor<+> Kif3a/Kif3a Tg(Pkhd1-cre)1Igr/?
Background: involves: 129S1/Sv * 129S4/SvJaeSor * 129X1/SvJ
Zygosity: cn
Has Mutant Allele: true
Publication
First Author: Shibazaki S
Year: 2008
Journal: Hum Mol Genet
Title: Cyst formation and activation of the extracellular regulated kinase pathway after kidney specific inactivation of Pkd1.
Volume: 17
Issue: 11
Pages: 1505-16
Publication
First Author: Patel V
Year: 2008
Journal: Hum Mol Genet
Title: Acute kidney injury and aberrant planar cell polarity induce cyst formation in mice lacking renal cilia.
Volume: 17
Issue: 11
Pages: 1578-90
Publication
First Author: Lakhia R
Year: 2016
Journal: J Am Soc Nephrol
Title: MicroRNA-21 Aggravates Cyst Growth in a Model of Polycystic Kidney Disease.
Volume: 27
Issue: 8
Pages: 2319-30
Publication
First Author: Ma M
Year: 2013
Journal: Nat Genet
Title: Loss of cilia suppresses cyst growth in genetic models of autosomal dominant polycystic kidney disease.
Volume: 45
Issue: 9
Pages: 1004-12
Gene
Type: gene
Organism: Rattus norvegicus
Publication
First Author: Nagasawa Y
Year: 2002
Journal: J Am Soc Nephrol
Title: Identification and characterization of Pkhd1, the mouse orthologue of the human ARPKD gene.
Volume: 13
Issue: 9
Pages: 2246-58
Publication
First Author: Xiong H
Year: 2002
Journal: Genomics
Title: A novel gene encoding a TIG multiple domain protein is a positional candidate for autosomal recessive polycystic kidney disease.
Volume: 80
Issue: 1
Pages: 96-104
Publication
First Author: Moser M
Year: 2005
Journal: Hepatology
Title: A mouse model for cystic biliary dysgenesis in autosomal recessive polycystic kidney disease (ARPKD).
Volume: 41
Issue: 5
Pages: 1113-21
Publication
First Author: Gallagher AR
Year: 2008
Journal: Am J Pathol
Title: Biliary and pancreatic dysgenesis in mice harboring a mutation in Pkhd1.
Volume: 172
Issue: 2
Pages: 417-29
Publication
First Author: Williams SS
Year: 2008
Journal: Pediatr Nephrol
Title: Kidney cysts, pancreatic cysts, and biliary disease in a mouse model of autosomal recessive polycystic kidney disease.
Volume: 23
Issue: 5
Pages: 733-41
Publication
First Author: Wang S
Year: 2007
Journal: Mol Cell Biol
Title: Fibrocystin/polyductin, found in the same protein complex with polycystin-2, regulates calcium responses in kidney epithelia.
Volume: 27
Issue: 8
Pages: 3241-52
Publication
First Author: Kim I
Year: 2008
Journal: J Am Soc Nephrol
Title: Fibrocystin/polyductin modulates renal tubular formation by regulating polycystin-2 expression and function.
Volume: 19
Issue: 3
Pages: 455-68
Publication
First Author: Garcia-Gonzalez MA
Year: 2007
Journal: Hum Mol Genet
Title: Genetic interaction studies link autosomal dominant and recessive polycystic kidney disease in a common pathway.
Volume: 16
Issue: 16
Pages: 1940-50
Publication
First Author: Hossain Z
Year: 2007
Journal: Proc Natl Acad Sci U S A
Title: Glomerulocystic kidney disease in mice with a targeted inactivation of Wwtr1.
Volume: 104
Issue: 5
Pages: 1631-6
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus caroli
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus musculus
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus musculus
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus musculus
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus musculus
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus musculus
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus musculus
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus musculus
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus musculus
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus musculus
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus musculus
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus musculus
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus musculus
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus musculus
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus musculus
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus musculus
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus musculus
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus pahari
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus spretus
Publication
First Author: Woollard JR
Year: 2007
Journal: Kidney Int
Title: A mouse model of autosomal recessive polycystic kidney disease with biliary duct and proximal tubule dilatation.
Volume: 72
Issue: 3
Pages: 328-36
GXD Expression    
Probe: MGI:4365600
Assay Type: Western blot
Annotation Date: 2009-10-27
Strength: Present
Sex: Not Specified
Emaps: EMAPS:1672828
Stage: TS28
Assay Id: MGI:4365604
Age: postnatal adult
Image: 1
Detected: true
Specimen Num: 4
GXD Expression    
Probe: MGI:4365600
Assay Type: Western blot
Annotation Date: 2009-10-27
Strength: Present
Sex: Not Specified
Emaps: EMAPS:1610528
Stage: TS28
Assay Id: MGI:4365604
Age: postnatal adult
Image: 1
Detected: true
Specimen Num: 3
GXD Expression      
Probe: MGI:4939238
Assay Type: RT-PCR
Annotation Date: 2011-03-02
Strength: Present
Sex: Not Specified
Emaps: EMAPS:1737326
Stage: TS26
Assay Id: MGI:4939437
Age: embryonic day 18.5
Detected: true
Specimen Num: 2
GXD Expression      
Probe: MGI:4939238
Assay Type: RT-PCR
Annotation Date: 2011-03-02
Strength: Present
Sex: Not Specified
Emaps: EMAPS:1737326
Stage: TS26
Assay Id: MGI:4939437
Age: embryonic day 18.5
Detected: true
Specimen Num: 1
GXD Expression    
Probe: MGI:4365600
Assay Type: Western blot
Annotation Date: 2009-10-27
Strength: Present
Sex: Not Specified
Emaps: EMAPS:1737328
Stage: TS28
Assay Id: MGI:4365604
Age: postnatal adult
Image: 1
Detected: true
Specimen Num: 1
GXD Expression    
Probe: MGI:4365600
Assay Type: Western blot
Annotation Date: 2009-10-27
Strength: Present
Sex: Not Specified
Emaps: EMAPS:1684628
Stage: TS28
Assay Id: MGI:4365604
Age: postnatal adult
Image: 1
Detected: true
Specimen Num: 2
GXD Expression    
Probe: MGI:4365594
Assay Type: Immunohistochemistry
Annotation Date: 2009-10-27
Strength: Absent
Sex: Not Specified
Emaps: EMAPS:2768123
Stage: TS23
Assay Id: MGI:4365639
Age: embryonic day 15.5
Image: 2H
Detected: false
Specimen Num: 6
GXD Expression
Probe: MGI:4365594
Assay Type: Immunohistochemistry
Annotation Date: 2009-10-27
Strength: Present
Sex: Not Specified
Emaps: EMAPS:1684623
Pattern: Regionally restricted
Stage: TS23
Assay Id: MGI:4365639
Age: embryonic day 15.5
Image: 2H
Note: Expression is in immature hepatocytes.
Detected: true
Specimen Num: 6
GXD Expression
Probe: MGI:4365594
Assay Type: Immunohistochemistry
Annotation Date: 2009-10-27
Strength: Present
Sex: Not Specified
Emaps: EMAPS:1684623
Pattern: Regionally restricted
Stage: TS23
Assay Id: MGI:4365639
Age: embryonic day 15.5
Image: 2J
Note: Expression is in immature hepatocytes.
Detected: true
Specimen Num: 8
GXD Expression
Probe: MGI:4365594
Assay Type: Immunohistochemistry
Annotation Date: 2009-10-27
Strength: Present
Sex: Not Specified
Emaps: EMAPS:1684623
Pattern: Regionally restricted
Stage: TS23
Assay Id: MGI:4365639
Age: embryonic day 15.5
Image: 2I
Note: Expression is in immature hepatocytes.
Detected: true
Specimen Num: 7
GXD Expression  
Probe: MGI:929510
Assay Type: RNA in situ
Annotation Date: 2010-09-14
Strength: Moderate
Sex: Not Specified
Emaps: EMAPS:1867623
Pattern: Regionally restricted
Stage: TS23
Assay Id: MGI:4827229
Age: embryonic day 14.5
Image: euxassay_006196_18
Detected: true
Specimen Num: 18
GXD Expression  
Probe: MGI:929510
Assay Type: RNA in situ
Annotation Date: 2010-09-14
Strength: Moderate
Sex: Not Specified
Emaps: EMAPS:1867623
Pattern: Regionally restricted
Stage: TS23
Assay Id: MGI:4827229
Age: embryonic day 14.5
Image: euxassay_006196_17
Detected: true
Specimen Num: 17
GXD Expression  
Probe: MGI:929510
Assay Type: RNA in situ
Annotation Date: 2010-09-14
Strength: Moderate
Sex: Not Specified
Emaps: EMAPS:1867623
Pattern: Regionally restricted
Stage: TS23
Assay Id: MGI:4827229
Age: embryonic day 14.5
Image: euxassay_006196_16
Detected: true
Specimen Num: 16
GXD Expression  
Probe: MGI:929510
Assay Type: RNA in situ
Annotation Date: 2010-09-14
Strength: Moderate
Sex: Not Specified
Emaps: EMAPS:1867623
Pattern: Regionally restricted
Stage: TS23
Assay Id: MGI:4827229
Age: embryonic day 14.5
Image: euxassay_006196_15
Detected: true
Specimen Num: 15
GXD Expression  
Probe: MGI:929510
Assay Type: RNA in situ
Annotation Date: 2010-09-14
Strength: Moderate
Sex: Not Specified
Emaps: EMAPS:1867623
Pattern: Regionally restricted
Stage: TS23
Assay Id: MGI:4827229
Age: embryonic day 14.5
Image: euxassay_006196_14
Detected: true
Specimen Num: 14
GXD Expression  
Probe: MGI:929510
Assay Type: RNA in situ
Annotation Date: 2010-09-14
Strength: Moderate
Sex: Not Specified
Emaps: EMAPS:1867623
Pattern: Regionally restricted
Stage: TS23
Assay Id: MGI:4827229
Age: embryonic day 14.5
Image: euxassay_006196_13
Detected: true
Specimen Num: 13
GXD Expression  
Probe: MGI:929510
Assay Type: RNA in situ
Annotation Date: 2010-09-14
Strength: Moderate
Sex: Not Specified
Emaps: EMAPS:1867623
Pattern: Regionally restricted
Stage: TS23
Assay Id: MGI:4827229
Age: embryonic day 14.5
Image: euxassay_006196_08
Detected: true
Specimen Num: 8
GXD Expression  
Probe: MGI:929510
Assay Type: RNA in situ
Annotation Date: 2010-09-14
Strength: Moderate
Sex: Not Specified
Emaps: EMAPS:1867623
Pattern: Regionally restricted
Stage: TS23
Assay Id: MGI:4827229
Age: embryonic day 14.5
Image: euxassay_006196_07
Detected: true
Specimen Num: 7
GXD Expression  
Probe: MGI:929510
Assay Type: RNA in situ
Annotation Date: 2010-09-14
Strength: Moderate
Sex: Not Specified
Emaps: EMAPS:1867623
Pattern: Regionally restricted
Stage: TS23
Assay Id: MGI:4827229
Age: embryonic day 14.5
Image: euxassay_006196_06
Detected: true
Specimen Num: 6
GXD Expression  
Probe: MGI:929510
Assay Type: RNA in situ
Annotation Date: 2010-09-14
Strength: Moderate
Sex: Not Specified
Emaps: EMAPS:1867623
Pattern: Regionally restricted
Stage: TS23
Assay Id: MGI:4827229
Age: embryonic day 14.5
Image: euxassay_006196_05
Detected: true
Specimen Num: 5
GXD Expression  
Probe: MGI:929510
Assay Type: RNA in situ
Annotation Date: 2010-09-14
Strength: Moderate
Sex: Not Specified
Emaps: EMAPS:1867623
Pattern: Regionally restricted
Stage: TS23
Assay Id: MGI:4827229
Age: embryonic day 14.5
Image: euxassay_006196_04
Detected: true
Specimen Num: 4
GXD Expression  
Probe: MGI:929510
Assay Type: RNA in situ
Annotation Date: 2010-09-14
Strength: Moderate
Sex: Not Specified
Emaps: EMAPS:1867623
Pattern: Regionally restricted
Stage: TS23
Assay Id: MGI:4827229
Age: embryonic day 14.5
Image: euxassay_006196_03
Detected: true
Specimen Num: 3