anti-Polycystic Kidney and Hepatic Disease 1 Protein (PKHD1) Antikörper

The protein encoded by PKHD1 is predicted to have a single transmembrane (TM)-spanning domain and multiple copies of an immunoglobulin-like plexin-transcription-factor domain. Zusätzlich bieten wir Ihnen und viele weitere Produktgruppen zu diesem Protein an.

Alle Antikörper anzeigen Gen GeneID UniProt
PKHD1 5314 P08F94
PKHD1 241035  
Anti-Ratte PKHD1 PKHD1 301287  
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Showing 10 out of 40 products:

Katalog Nr. Reaktivität Wirt Konjugat Applikation Bilder Menge Anbieter Lieferzeit Preis Details
Human Kaninchen Unkonjugiert ELISA Immunocytochemistry/Immunofluorescence: PKHD1 Antibody [BC110-60480] - analysis of PKHD1 in mouse inner medullary collecting duct cell line using anti-PKHD1 antibody. Image from verified customer review. 0.1 mL Anmelden zum Anzeigen 7 bis 9 Tage
$444.19
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Human Kaninchen Unkonjugiert ELISA   0.1 mL Anmelden zum Anzeigen 7 bis 9 Tage
$444.19
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Human Kaninchen Unkonjugiert ELISA, ICC, IF   0.1 mL Anmelden zum Anzeigen 7 bis 9 Tage
$444.19
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Human Kaninchen Unkonjugiert ELISA   0.1 mL Anmelden zum Anzeigen 7 bis 9 Tage
$444.19
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Human Kaninchen Unkonjugiert IHC, IHC (p) Immunohistochemistry: PKHD1 Antibody [NBP1-89003] - Staining of human kidney shows strong cytoplasmic positivity in cells in tubules. Immunohistochemistry: PKHD1 Antibody [NBP1-89003] - Staining of human liver shows strong cytoplasmic positivity in hepatocytes. 0.1 mL Anmelden zum Anzeigen 10 bis 13 Tage
$491.63
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Human Maus Unkonjugiert ELISA, FACS, ICC, IHC Black line: Control Antigen (100 ng); Purple line: Antigen(10ng); Blue line: Antigen (50 ng); Red line: Antigen (100 ng); Western blot analysis using PKHD1 mAb against mouse PKHD1(AA: 3878-4060) recombinant protein. (Expected MW is 23 kDa) 100 μg Anmelden zum Anzeigen 2 bis 3 Tage
$430.10
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Human Kaninchen Unkonjugiert ELISA   100 μL Anmelden zum Anzeigen 7 bis 9 Tage
$654.50
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Human Kaninchen Unkonjugiert ELISA   100 μL Anmelden zum Anzeigen 7 bis 9 Tage
$654.50
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Human Kaninchen FITC ELISA   0.1 mL Anmelden zum Anzeigen 7 bis 9 Tage
$556.31
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Human Kaninchen FITC ELISA   0.1 mL Anmelden zum Anzeigen 7 bis 9 Tage
$556.31
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PKHD1 Antikörper nach Reaktivität, Anwendung, Klonalität and Konjugat

Attribute Applikationen Wirt Klonalität Konjugat
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Weitere Antikörper gegen PKHD1 Interaktionspartner

Human Polycystic Kidney and Hepatic Disease 1 Protein (PKHD1) Interaktionspartner

  1. SEven novel mutations in PKHD1 gene were identified in 15 Chinese families with polycystic kidney disease.

  2. This report expands the spectrum of PKHD1 mutations in probands with autosomal recessive polycystic kidney disease and confirms the allelic heterogeneity of this disorder.

  3. In this study, the entire PKHD1 coding region was amplified by 29 reactions to avoid the specific PCR amplification of individual exons, which generated the size of 1 to 7 kb products by LR PCR. This method was compared to the screening method with standard direct sequencing of each individual exon of the gene by a reference laboratory in 15 patients with ARPKD

  4. Data show that the compound heterozygous mutations of c.11314C>T from mother and a missense c.889T>A from father of the polycystic kidney and hepatic disease 1 protein (PKHD1) gene were identified in the fetus.

  5. Compound heterozygous PKHD1 variants cause a wide spectrum of ductal plate malformations.

  6. Results identified six novel mutations (PKHD1: p.Thr777Met, p.Tyr2260Cys; ABCB11 (zeige ABCB11 Antikörper): p.Val1112Phe, c.611+1G > A, p.Gly628Trpfs*3 and NPC1 (zeige NPC1 Antikörper): p.Glu391Lys) for the diagnostic of inherited infantile cholestatic disorders.

  7. Data indicate that seventeen different polycystic kidney and hepatic disease 1 (autosomal recessive) protein (PKHD1) mutations (5 novel) were detected, including deletion of one exon.

  8. Our data provide strong evidence that the p.M627K substitution at the PKHD1 locus is a founder mutation for Autosomal recessive polycystic kidney disease in the Afrikaner population

  9. A novel c.9059T>C mutation in PKHD1 gene expands mutation spectrum for autosomal recessive polycystic kidney disease.

  10. Both polycystins were detected on the spindle and mid-body of mitotic cells, while fibrocystin was on centrosome throughout cell cycle.

Mouse (Murine) Polycystic Kidney and Hepatic Disease 1 Protein (PKHD1) Interaktionspartner

  1. this study shows that loss of functional Pkhd1 on the NOD background produces early bile duct abnormalities, initiating a break in tolerance that leads to autoimmune cholangitis in NOD.Abd3 congenic mice

  2. fibrocystin-defective cholangiocytes are characterized by a beta (zeige SUCLA2 Antikörper)-catenin (zeige CTNNB1 Antikörper)-dependent secretion of a range of chemokines which stimulate bone marrow-derived macrophage recruitment

  3. Intragenic motifs regulate the transcriptional complexity of Pkhd1/PKHD1

  4. Mutations in the Pkhd1 gene result in autosomal recessive polycystic kidney disease (ARPKD) in humans.

  5. PKHD1 localizes to the mitotic spindle and regulates spindle bipolarity.

  6. Loss of oriented cell division is a feature of Pkhd1 mutation and cyst formation, but it is neither sufficient to produce kidney cysts nor required to initiate cyst formation after mutation in Pkd1 (zeige PKD1 Antikörper) or Pkd2 (zeige PKD2 Antikörper).

  7. key features of human PKHD1 are highly conserved in the mouse suggesting that the complicated pattern of splicing is likely to be functionally important.

  8. In cultured renal cells, the PKHD1 gene product colocalized with polycystin-2 (zeige PKD2 Antikörper), the gene product of autosomal dominant polycystic disease type 2.

  9. the hepatocyte nuclear factor-1beta (HNF-1beta (zeige HNF1B Antikörper)) C-terminal domain has a role in Pkhd1 (ARPKD) gene transcription and renal cystogenesis

  10. The role of polyductin in liver and kidney may be functionally divergent, because protein domains essential for bile duct development do not affect nephrogenesis in our mouse model.

PKHD1 Antigen-Profil

Protein Überblick

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.

Genbezeichner und Symbole assoziert mit PKHD1

  • PKHD1, fibrocystin/polyductin (PKHD1) Antikörper
  • polycystic kidney and hepatic disease 1 (Pkhd1) Antikörper
  • AI118496 Antikörper
  • AI182499 Antikörper
  • ARPKD Antikörper
  • FCYT Antikörper
  • FPC Antikörper
  • Tigm1 Antikörper

Bezeichner auf Proteinebene für PKHD1

TIG multiple domains 1 , fibrocystin , polycystic kidney and hepatic disease 1 protein , polyductin , tigmin , polycystic kidney and hepatic disease 1 (autosomal recessive) , polycystic kidney and hepatic disease 1 homolog

GENE ID SPEZIES
5314 Homo sapiens
241035 Mus musculus
301287 Rattus norvegicus
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