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Fibrocystin

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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.

Below are the list of possible Fibrocystin products. If you cannot find the target and/or product is not available in our catalog, please click here to contact us and request the product or submit your request for custom elisa kit production, custom recombinant protein production or custom antibody production. Custom ELISA Kits, Recombinant Proteins and Antibodies can be designed, manufactured and produced according to the researcher's specifications.
 

Fibrocystin

 Fibrocystin ELISA Kit
 Fibrocystin Recombinant
 Fibrocystin Antibody
Also known as Fibrocystin (Polycystic kidney and hepatic disease 1 protein) (Polyductin) (Tigmin).
PKHD1: 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.
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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. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Cell surface; Membrane protein, integral

Chromosomal Location of Human Ortholog: 6p12.2

Cellular Component: anchored to external side of plasma membrane; apical plasma membrane; centrosome; cilium; cytoplasm; perinuclear region of cytoplasm

Molecular Function: protein binding

Biological Process: cell-cell adhesion; cellular calcium ion homeostasis; inhibition of NF-kappaB transcription factor; kidney development; negative regulation of apoptosis; negative regulation of cell motility; negative regulation of protein kinase B signaling cascade; positive regulation of cell proliferation; regulation of TOR signaling pathway

Disease: Polycystic Kidney Disease, Autosomal Recessive
 PKHD1 ELISA Kit
 PKHD1 Recombinant
 PKHD1 Antibody
 FCYT ELISA Kit
 FCYT Recombinant
 FCYT Antibody
 TIGM1 ELISA Kit
 TIGM1 Recombinant
 TIGM1 Antibody
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Fibrocystin-L

 Fibrocystin-L ELISA Kit
 Fibrocystin-L Recombinant
 Fibrocystin-L Antibody
Also known as Fibrocystin-L (Polycystic kidney and hepatic disease 1-like protein 1) (PKHD1-like protein 1).
PKHD1L1:

Protein type: Membrane protein, integral

Chromosomal Location of Human Ortholog: 8q23

Cellular Component: cytosol
 PKHD1L1 ELISA Kit
 PKHD1L1 Recombinant
 PKHD1L1 Antibody
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