Mutations in the PKHD1 gene, which encodes fibrocystin, cause autosomal recessive polycystic kidney disease (ARPKD). Unfortunately, the lack of specific antibodies to the mouse protein impairs the study of splicing, post-translational processing, shedding, and temporal and spatial expression of endogenous fibrocystin at the cellular and subcellular level.
Aliases : FPC; Tigm1; AI118496; AI182499
Entrez GeneID: 241035
Swissprot: E9PZ36
WB Predicted band size: 445kDa
Host/Isotype: Rat IgG1
Species Reactivity: Human
Immunogen: Purified recombinant fragment of mouse PKHD1 (AA: 3878-4060) expressed in E. Coli.