Fibrocystin Antibodies

Santa Cruz Biotechnology, Inc. offers a broad range of Fibrocystin antibodies. Select Fibrocystin antibodies from several monoclonal and/or polyclonal Fibrocystin antibodies listed below. View detailed Fibrocystin antibody specifications by linking to the specific product blocks. Select appropriate Fibrocystin antibodies for your research by isotype, epitope, applications and species reactivity. Fibrocystin gene silencer products in siRNA, shRNA Plasmid and shRNA Lentiviral Particle formats are also available.

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Also see additional Fibrocystin Antibodies including, Fibrocystin L

Fibrocystin is a type I membrane protein that undergoes regulated proteolysis. Many proteolytic cleavages occur on the ectodomain whereas at least one cleavage occurs on the cytoplasmic portion. Fibrocystin may participate in the mediation of intracellular calcium in the distal nephron in a manner similar to PKD1 and PKD2. Mutations in the PKHD1 gene, which encodes fibrocystin, result in autosomal recessive polycystic kidney disease (ARPKD), a severe form of polycystic kidney disease characterized by enlarged kidneys and congenital hepatic fibrosis. A related protein, Fibrocystin L, also designated polycystic kidney and hepatic disease 1-like protein 1 or PKHD1L1, shares 41% similarity with Fibrocystin in the extracellular domain, but is not associated with ARPKD. Fibrocystin L is a large receptor protein with a signal peptide, a single transmembrane domain and a short cytoplasmic tail. It is ubiquitously expressed at low levels, with higher expression in spleen and thymus as well as in activated T-cells and B lymphoblasts, suggesting a role for Fibrocystin L in the immune response.