IQCB1 Antibody

Code CSB-PA003400
Size US$167
Image
  • Western Blot analysis of SH-SY5Y cells using Nephrocystin-5 Polyclonal Antibody
  • Western Blot analysis of 293 Jurkat cells using Nephrocystin-5 Polyclonal Antibody
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Product Details

Uniprot No. Q15051
Target Names IQCB1
Alternative Names IQ calmodulin-binding motif containing 1 antibody; IQ calmodulin-binding motif-containing protein 1 antibody; IQ motif containing B1 antibody; Iqcb1 antibody; IQCB1_HUMAN antibody; KIAA0036 antibody; Nephrocystin-5 antibody; NPHP5 antibody; p53 and DNA damage-regulated IQ motif protein antibody; SLSN5 antibody
Raised in Rabbit
Species Reactivity Human
Immunogen Synthesized peptide derived from the Internal region of Human Nephrocystin-5.
Immunogen Species Homo sapiens (Human)
Conjugate Non-conjugated
Isotype IgG
Purification Method The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Concentration It differs from different batches. Please contact us to confirm it.
Buffer Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Form Liquid
Tested Applications WB, IHC, ELISA
Recommended Dilution
Application Recommended Dilution
WB 1:500-1:2000
IHC 1:100-1:300
ELISA 1:40000
Protocols Western Blotting(WB) Protocol
Immunohistochemistry (IHC) Protocol
ELISA Protocol
Troubleshooting and FAQs Antibody FAQs
Storage Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

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Target Data

Function Involved in ciliogenesis. The function in an early step in cilia formation depends on its association with CEP290/NPHP6
Gene References into Functions
  1. Study demonstrates the interaction between CNNM4 and IQCB1, which provides the first link between CNNM4 and IQCB1 that causes Leber congenital amaurosis and retinal dystrophy when mutated, providing important insights into the molecular pathogenic mechanisms of retinal dystrophy in Jalili syndrome. PMID: 29322253
  2. that nephrocystin-5 is essential for photoreceptor outer segment formation PMID: 27328943
  3. Dynamic ubiquitination and deubiquitination of NPHP5 plays a crucial role in the regulation of ciliogenesis. NPHP5 directly binds to a deubiquitinating enzyme USP9X/FAM and two E3 ubiquitin ligases BBS11/TRIM32 and MARCH7/axotrophin. PMID: 28498859
  4. NPHP5-mutant dogs recapitulate the human phenotype of very early loss of rods, and relative retention of the central retinal cone photoreceptors that lack function. PMID: 27506978
  5. NPHP5 and Cep290 regulate BBSome integrity, ciliary trafficking and cargo delivery. PMID: 25552655
  6. High-throughput mutation analysis identified a homozygous truncating mutation (c.1504C>T, p.R502*) in the NPHP5 in 5 families in Iranian children with nephronophthisis. PMID: 25851290
  7. mutation is predicted to introduce a new open reading frame that results in the truncation of the C-terminal 235 amino acids of nephrocystin-5 and its consequent loss of function PMID: 24674142
  8. NPHP5 mutations impair protein interaction with Cep290 and localize to centrosomes, thereby compromising cilia formation. PMID: 23446637
  9. Genetic variation may affect severity of disease for X-linked retinitis pigmentosa. PMID: 22183348
  10. Data show that the minor allele (N) of I393N in IQCB1 and the common allele (R) of R744Q in RPGRIP1L were associated with severe disease in XlRP with RPGR mutations. PMID: 21857984
  11. Cone photoreceptors are the main targets for gene therapy of NPHP5 (IQCB1) or NPHP6 (CEP290) blindness: generation of an all-cone Nphp6 hypomorph mouse that mimics the human retinal ciliopathy. PMID: 21245082
  12. Results show that the onset of renal failure in patients with IQCB1 mutations is highly variable, and that mutations are also found in Leber congenital amaurosis (LCA) patients without nephronophthisis, rendering IQCB1 a new gene for LCA. PMID: 20881296
  13. Mutations in NPHP5 can cause Leber congenital amaurosis (LCA)without early-onset renal disease. PMID: 21220633
  14. nephrocystin-5, RPGR and calmodulin can be coimmunoprecipitated from retinal extracts, and that these proteins localize to connecting cilia of photoreceptors and to primary cilia of renal epithelial cells PMID: 15723066

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Involvement in disease Senior-Loken syndrome 5 (SLSN5); Leber congenital amaurosis 10 (LCA10)
Subcellular Location Cytoplasm, cytoskeleton, microtubule organizing center, centrosome, Cytoplasm, cytoskeleton, microtubule organizing center, centrosome, centriole
Tissue Specificity 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.
Database Links

HGNC: 28949

OMIM: 609237

KEGG: hsa:9657

STRING: 9606.ENSP00000311505

UniGene: Hs.604110

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