Target：Nephrocystin-5

Gene Name：IQCB1

Protein Name：IQ calmodulin-binding motif-containing protein 1

Human Gene Id：9657

Human Swiss Prot No：Q15051

Mouse Swiss Prot No：Q8BP00

Immunogen：The antiserum was produced against synthesized peptide derived from human IQCB1. AA range:431-480

Specificity：Nephrocystin-5 Polyclonal Antibody detects endogenous levels of Nephrocystin-5 protein.

Formulation：Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

Source：Polyclonal, Rabbit,IgG

Dilution：WB 1:500 - 1:2000. IHC 1:100 - 1:300. ELISA: 1:40000.. IF 1:50-200

Purification：The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Concentration：1 mg/ml

Storage Stability：-15°C to -25°C/1 year(Do not lower than -25°C)

Other Name：IQCB1;KIAA0036;NPHP5;OK/SW-cl.85;IQ calmodulin-binding motif-containing protein 1;Nephrocystin-5;p53 and DNA damage-regulated IQ motif protein;PIQ

Observed Band(KD)：69kD

Background： This gene encodes a nephrocystin protein that interacts with calmodulin and the retinitis pigmentosa GTPase regulator protein. The encoded protein has a central coiled-coil region and two calmodulin-binding IQ domains. It is localized to the primary cilia of renal epithelial cells and connecting cilia of photoreceptor cells. The protein is thought to play a role in ciliary function. Defects in this gene result in Senior-Loken syndrome type 5. Alternative splicing results in multiple transcript variants. A pseudogene of this gene is found on chromosome 6. [provided by RefSeq, Jan 2016],

Function：disease:Defects in IQCB1 are the cause of Senior-Loken syndrome type 5 (SLSN5) [MIM:609254]. SLSN is a renal-retinal disorder, characterized by progressive wasting of the filtering unit of the kidney (nephronophthisis), with or without medullary cystic renal disease, and progressive eye disease. Typically this disorder becomes apparent during the first year of life.,similarity:Contains 4 IQ domains.,subunit:Interacts with calmodulin.,tissue specificity:Ubiquitously expressed in fetal and adult tissues. Localized to the outer segments and connecting cilia of photoreceptor cells.,

Subcellular Location：Cytoplasm, cytoskeleton, microtubule organizing center, centrosome . Cytoplasm, cytoskeleton, microtubule organizing center, centrosome, centriole . Localization to the centrosome depends on the interaction with CEP290/NPHP6.

Expression：Ubiquitously expressed in fetal and adult tissues. Localized to the outer segments and connecting cilia of photoreceptor cells. Up-regulated in a number of primary colorectal and gastric tumors.