Product Datasheet  
PKHD1 Antibody  
Catalog Number: 48398  
Technical:tech@swbio.com  
Information:info@swbio.com  
Description  
  • host_species：  
  • Mouse
  • Amount：  
  • 100μgμg
  • Swiss-Prot No.：  
  • Swiss-Prot#:P08F94
  • Form of Antibody：  
  • 1*TBS (pH7.4), 1%BSA, 40%Glycerol. Preservative: 0.05% Sodium Azide.
  • Storage：  
  • Store at -20˚C
  • Immunogen：  
  • Recombinant protein
  • reactivity：  
  • Hu
  • appl_detail：  
  • WB: 1:500-1:2,000
    IHC: 1:50-1:200
    ICC: 1:50-1:200

    FC: 1:50-1:100
  • other_names：  
  • ARPKD antibody
    FCYT antibody
    Fibrocystin antibody
    FPC antibody
    PKHD1 antibody
    PKHD1_HUMAN antibody
    Polycystic kidney and hepatic disease 1 protein antibody
    Polyductin antibody
    TIGM1 antibody
    Tigmin antibody
  • Purification：  
  • ProA affinity purified
  • Specificity：  
  • Applications：  
  • WB, ICC, IHC, FC
  • Background：  
  • May be required for correct bipolar cell division through the regulation of centrosome duplication and mitotic spindle assembly. May be a receptor protein that acts in collecting-duct and biliary differentiation. Defects in PKHD1 are the cause of polycystic kidney disease autosomal recessive (ARPKD). ARPKD is a severe form of polycystic kidney disease affecting the kidneys and the hepatic biliary tract. The clinical spectrum is widely variable, with most cases presenting during infancy. The fetal phenotypic features classically include enlarged and echogenic kidneys, as well as oligohydramnios secondary to a poor urine output. Up to 50% of the affected neonates die shortly after birth, as a result of severe pulmonary hypoplasia and secondary respiratory insufficiency. In the subset that survives the perinatal period, morbidity and mortality are mainly related to severe systemic hypertension, renal insufficiency, and portal hypertension due to portal-tract fibrosis.



 
© Signalway Biotechnology All Rights Reserved.