Anti-Nephrocystin-5 Rabbit Polyclonal Antibody
Product Information
Downloads & Supports
Target
-
IQCB1
Host
Storage
-
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
Clonality
Clone Number
-
-
Isotype
-
IgG
Reactivity
Application
Application Details
-
WB: 1/500-1/1000 IHC: 1/50-1/200
Immunogen
-
Synthetic peptide corresponding to Human Nephrocystin-5.
Molecular Weight
-
Calculated MW: 69kDa
Formulation
-
1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2.
Form
-
Liquid
Gene ID
-
9657
Uniprot ID
-
Q15051
Background
-
The nephrocystin proteins comprise a family of five enzymes that commonly interact with p130Cas, proline-rich tyrosine kinases, calmodulin and tensin, indicating that these proteins may participate in a common signaling pathway. Localized to the outer segments and primary cilia of photoreceptor cells, nephrocystin-5 is complexed with RPGR (retinitis pigmentosa GTPase regulator) and interacts directly with calmodulin. Nephrocystin-5 is thought to participate with RPGR in a pathway of ciliary function in the kidey and retina. Mutations in the gene encoding nephrocystin-5 are the primary cause of Senior-Loken syndrome 5, a juvenile disorder characterized by defects in the waste filtering system of the kidney, as well as retinal degradation.
Research Area
-
-
Synonyms
-
IQ calmodulin-binding motif-containing protein 1; Nephrocystin-5; p53 and DNA damage-regulated IQ motif protein; PIQ; IQCB1; KIAA0036; NPHP5; OK/SW-cl.85