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  4. SPTAN1 Recombinant Monoclonal Antibody

SPTAN1 Recombinant Monoclonal Antibody

  • 中文名稱：
    SPTAN1 Recombinant Monoclonal Antibody
  • 貨號：
    CSB-RA142176A0HU
  • 規格：
    ￥1320
  • 圖片：
    • IHC image of CSB-RA142176A0HU diluted at 1:100 and staining in paraffin-embedded human small intestine tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a Goat anti-rabbit polymer IgG labeled by HRP and visualized using 0.05% DAB.
    • Overlay Peak curve showing Hela cells stained with CSB-RA142176A0HU (red line) at 1:100. The cells were fixed in 4% formaldehyde and permeated by 0.2% TritonX-100 for?10min. Then 10% normal goat serum to block non-specific protein-protein interactions followed by the antibody (1ug/1*106cells) for 45min at 4℃. The secondary antibody used was FITC-conjugated goat anti-rabbit IgG (H+L) at 1/200 dilution for 35min at 4℃.Control antibody (green line) was Rabbit IgG (1ug/1*106cells) used under the same conditions. Acquisition of >10,000 events was performed.
  • 其他：

產品詳情

  • Uniprot No.：
  • 基因名：
  • 別名：
    Spectrin alpha chain, non-erythrocytic 1 (Alpha-II spectrin) (Fodrin alpha chain) (Spectrin, non-erythroid alpha subunit), SPTAN1, NEAS SPTA2
  • 反應種屬：
    Human
  • 免疫原：
    A synthesized peptide derived from Human SPTAN1 protein
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標記方式：
    Non-conjugated
  • 克隆類型：
    Monoclonal
  • 抗體亞型：
    Rabbit IgG
  • 純化方式：
    Affinity-chromatography
  • 克隆號：
    20E8
  • 濃度：
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液：
    Rabbit IgG in 10mM phosphate buffered saline , pH 7.4, 150mM sodium chloride, 0.05% BSA, 0.02% sodium azide and 50% glycerol.
  • 產品提供形式：
    Liquid
  • 應用范圍：
    ELISA, IHC, FC
  • 推薦稀釋比：
    Application Recommended Dilution
    IHC 1:50-1:200
    FC 1:20-1:200
  • Protocols：
  • 儲存條件：
    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.

產品評價

靶點詳情

  • 功能：
    Fodrin, which seems to be involved in secretion, interacts with calmodulin in a calcium-dependent manner and is thus candidate for the calcium-dependent movement of the cytoskeleton at the membrane.
  • 基因功能參考文獻：
    1. Spectrin may be engaged in regulation of distinct events necessary for the establishment and maturity of the immunological synapse. PMID: 29244882
    2. SPTAN1-related disorders comprise a wide spectrum of neurodevelopmental phenotypes ranging from mild to severe and progressive. Spectrin aggregate formation in fibroblasts with mutations in the alpha/beta heterodimerization domain seems to be associated with a severe neurodegenerative course. PMID: 29050398
    3. Alpha-spectrin is critical for recruitment of non-ubiquitinated FANCD2 to sites of damage, which has an important role in the repair response and interstrand cross-link repair. PMID: 26297932
    4. Studies demonstrate that alpha-IISp plays a critical role in maintaining chromosome stability in cells after DNA interstrand cross-links damage by repairing damage that occurs in both genomic and telomeric DNA. PMID: 25757157
    5. These results suggest that ubiquitin C-terminal hydrolase and alphaII-spectrin breakdown product 145 kDa may be useful in assessing outcome after pediatric traumatic brain injury. PMID: 22022780
    6. Variations in both alpha-spectrin (SPTA1) and beta-spectrin ( SPTB ) were found in a neonate with prolonged jaundice in a family where nine individuals had hereditary elliptocytosis. PMID: 24193021
    7. aggressiveness of MLH1-positive colorectal cancers might be related to SPTAN1. PMID: 24456667
    8. hypomyelination of the cerebral white matter was revealed at the age of 1 year, and a de novo heterozygous mutation in the SPTAN1 gene was confirmed PMID: 22656320
    9. loss of SPTAN1 switches TGF-beta signaling from tumor suppression to tumor promotion by engaging Notch signaling and activating SOX9 in esophageal adenocarcinoma. PMID: 23536563
    10. organization of a spectrin-like cytoskeleton is associated with keratinocyte differentiation, and cytoskeleton disruption is mediated by either PKCdelta(Thr505) phosphorylation associated with phosphorylated adducin or due to reduction of endogenous adducin PMID: 22163289
    11. In-frame mutations in the C-terminus of SPTAN1 cause a core set of manifestations that include severe intellectual disability, generalized epilepsy, and pontocerebellar atrophy. PMID: 22258530
    12. Analysis of alphaII-spectrin breakdown products in cerebrospinal fluid predicts mortality and injury severity in adults following traumatic brain injury. PMID: 20408766
    13. findings suggest that pathological aggregation of alpha/beta spectrin heterodimers and abnormal axon initial segment(AIS) integrity resulting from SPTAN1 mutations were involved in pathogenesis of infantile epilepsy PMID: 20493457
    14. Fanconi anemia proteins may play an important role in maintaining the stability of alphaIISp in the cell by regulating its cleavage by mu-calpain. PMID: 20518497
    15. This protein has been found differentially expressed in the anterior cingulate cortex from patients with schizophrenia PMID: 20381070
    16. SigA binds to epithelial HEp-2 cells as well as being able to induce fodrin degradation in vitro and in situ, further extending its documented role in the pathogenesis of Shigella infections. PMID: 20011051
    17. Review. 120 kDa alpha-fodrin is an important autoantigen in both animal models and Sjogren syndrome patients. Increased caspase cascade activity may be involved in the progression of alpha-fodrin proteolysis and tissue destruction. PMID: 12630566
    18. High affinity and slow overall kinetics of association/dissociation of alpha II-spectrin may suit it well to a role in strengthening cell junctions and providing stable anchorage for transmembrane proteins at points specified by cell-adhesion molecules. PMID: 12820899
    19. Fanconi anemia cell lines deficient in alphaII spectrin express normal levels of alphaII spectrin mRNA. PMID: 12893251
    20. These observations indicate that calpain is activated and reacts with alpha-fodrin as a substrate at the sarcolemma, and plays a key role in modulating sarcolemmal proteins to adapt to the specific conditions in each myopathy. PMID: 15948206
    21. The in vivo role of alpha-fodrin autoantigen in primary Sjogren's syndrome was analyzed. PMID: 16192640
    22. These studies indicate that alphaSpIISigma( *) may play a role in a number of diverse and important processes in the nucleus and that a deficiency in this protein, as occurs in Fanconi's anemia, could affect a number of critical cellular pathways. PMID: 16889989
    23. provide novel insights into spectrin functions by demonstrating the involvement of alphaII-spectrin in cell cycle regulation and actin organization PMID: 18978357
    24. The SH3 domain of SPTAN1 is a target for the Fanconi anemia protein, FANCG. PMID: 19102630
    25. depletion of alphaIISp in normal cells leads to a number of defects observed in Fanconi anemia cells, such as chromosome instability and a deficiency in cross-link repair. PMID: 19217883
    26. fodrin degradation occurs during galectin-1 T cell death and CD45 is essential for fodrin degradation to occur PMID: 19454697
    27. This protein has been found differentially expressed in the temporal lobe from patients with schizophrenia. PMID: 19034380

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  • 相關疾病：
    Epileptic encephalopathy, early infantile, 5 (EIEE5)
  • 亞細胞定位：
    Cytoplasm, cytoskeleton. Cytoplasm, cell cortex.
  • 蛋白家族：
    Spectrin family
  • 數據庫鏈接：

    HGNC: 11273

    OMIM: 182810

    KEGG: hsa:6709

    STRING: 9606.ENSP00000361824

    UniGene: Hs.372331



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