Western blot
All lanes: INVS antibody IgG at 14μg/ml + K562 whole cell lysate
Secondary
Goat polyclonal to rabbit IgG at 1/10000 dilution
Predicted band size: 118, 100, 11 kDa
Observed band size: 118 kDa
Immunohistochemistry of paraffin-embedded human liver cancer using CSB-PA011769LA01HU at dilution of 1:100
Immunofluorescent analysis of HepG2 cells using CSB-PA011769LA01HU at dilution of 1:100 and Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L)
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
貨期:
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.
用途:
For Research Use Only. Not for use in diagnostic or therapeutic procedures.
Required for normal renal development and establishment of left-right axis. Probably acts as a molecular switch between different Wnt signaling pathways. Inhibits the canonical Wnt pathway by targeting cytoplasmic disheveled (DVL1) for degradation by the ubiquitin-proteasome. This suggests that it is required in renal development to oppose the repression of terminal differentiation of tubular epithelial cells by Wnt signaling. Involved in the organization of apical junctions in kidney cells together with NPHP1, NPHP4 and RPGRIP1L/NPHP8. Does not seem to be strictly required for ciliogenesis.
基因功能參考文獻:
Collectively, these results indicated that inversin might promote the tumorigenicity of lung cancer cells and serve as a novel therapeutic target of non-small cell lung cancer. PMID: 28618971
Akt phosphorylates inversin at amino acids 864-866 that are required not only for Akt interaction, but also for inversin dimerization. PMID: 27220846
Truncating mutations in NPHP2 gene is associated with nephronophthisis-related ciliopathy. PMID: 23559409
INVS mutation can cause juvenile nephronophthisis with abnormal reactivity of the Wnt/beta-catenin pathway. PMID: 20798123
has conserved ankyrin repeat and IQ domains and interacts with calmodulin PMID: 11941489
analysis of a homozygous mutation in exon 13 of inversin (INVS) (C2719T, R907X) in a patient with retinitis pigmentosa and renal failure [case report] PMID: 16522655
Thus, although considered a common variant, inv(10)(p11.2q21.2) has a single ancestral founder among northern Europeans. PMID: 16642442
new mutant genes might be responsible for the early onset of ESRD in infantile NPHP with features of JBTS PMID: 17216245
Cystic kidney disease has been linked to mutations in the Invs gene in mice with an inversion of embryonic turning (inv/inv) and the INVS (NPHP2) gene in human infantile nephronophthisis (NPH). PMID: 18218308
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Nephronophthisis 2 (NPHP2)
亞細胞定位:
Cytoplasm. Cytoplasm, cytoskeleton. Cytoplasm, cytoskeleton, spindle. Membrane; Peripheral membrane protein. Nucleus. Cell projection, cilium. Note=Associates with several components of the cytoskeleton including ciliary, random and polarized microtubules. During mitosis, it is recruited to mitotic spindle. Frequently membrane-associated, membrane localization is dependent upon cell-cell contacts and is redistributed when cell adhesion is disrupted after incubation of the cell monolayer with low-calcium/EGTA medium.
組織特異性:
Widely expressed. Strongly expressed in the primary cilia of renal tubular cells.