SPTBN2 Antibody
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中文名稱(chēng)：SPTBN2兔多克隆抗體
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貨號(hào)：CSB-PA022636GA01HU
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規(guī)格：￥3,900
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其他：
產(chǎn)品詳情
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Uniprot No.：
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基因名：SPTBN2
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別名：SPTBN2 antibody; KIAA0302 antibody; SCA5Spectrin beta chain antibody; non-erythrocytic 2 antibody; Beta-III spectrin antibody; Spinocerebellar ataxia 5 protein antibody
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宿主：Rabbit
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反應(yīng)種屬：Human
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免疫原：Human SPTBN2
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免疫原種屬：Homo sapiens (Human)
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抗體亞型：IgG
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純化方式：Antigen Affinity purified
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濃度：It differs from different batches. Please contact us to confirm it.
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保存緩沖液：0.1M NaHCO3,0.1M Glycine, 0.02% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
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產(chǎn)品提供形式：Liquid
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應(yīng)用范圍：ELISA,WB
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Protocols：
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儲(chǔ)存條件：Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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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.
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用途：For Research Use Only. Not for use in diagnostic or therapeutic procedures.
產(chǎn)品評(píng)價(jià)
相關(guān)產(chǎn)品
靶點(diǎn)詳情
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功能：Probably plays an important role in neuronal membrane skeleton.
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基因功能參考文獻(xiàn)：
- the proposed methodology is validated against betaII-spectrin protein, a brain injury validated biomarker PMID: 28112201
- SCA5 missense mutation found in the spinocerebellar ataxia type 5 perturbs a closed-open structural equilibrium in the SCA5-actin-binding domain by lowering the energetic barrier between structural states. PMID: 29116080
- Cardiac beta2-spectrin and downstream molecules are regulated in multiple forms of cardiovascular disease via Ca(2+)- and calpain-dependent proteolysis. PMID: 27106045
- This study indicates that high-affinity actin binding of L253P beta-III-spectrin is a likely driver of neurodegeneration. PMID: 26883385
- beta2-Spectrin, a TGF-beta mediator and signaling molecule, is cleaved and activated by caspase-3/7, consequently enhancing apoptosis and transcriptional control to determine cell fate upon liver damage. PMID: 26884715
- TGF-beta/beta2-spectrin/CTCF-regulated tumor suppression in human stem cell disorder Beckwith-Wiedemann syndrome. PMID: 26784546
- investigated using either targeted next generation sequencing or trio-based exome sequencing and were found to have mutations in three different genes, KCNC3, ITPR1 and SPTBN2 PMID: 25981959
- First Japanese spinocerebellar ataxia type 5 (SCA5) family with a novel heterozygous three-nucleotide in-frame deletion mutation in the SPTBN2 gene. PMID: 25142508
- A homozygous SPTBN2 nonsense mutation underlying infantile ataxia and psychomotor delay in a human family. PMID: 23838597
- Mutant beta-III spectrin causes mislocalization and dysfunction of mGluR1alpha at dendritic spines. PMID: 25057192
- A novel missense mutation within a SPTBN2 spectrin repeat encoded by exon 12 was found in a family with spinocerebellar ataxia type 5. PMID: 22843192
- the identification of SPARCA1 and normal heterozygous carriers of the stop codon in SPTBN2 provides insights into the mechanism of molecular dominance in SCA5 and demonstrates that the cell-specific repertoire of spectrin subunits PMID: 23236289
- betaIII spectrin regulates the structural integrity and the secretory protein transport of the Golgi complex PMID: 23233669
- two gene markers (CNKSR3 and SPTBN2) differentiate between aspirin-exacerbated respiratory disease and aspirin-tolerant asthma with a perfect discriminative power PMID: 22457146
- This review summarizes data showing that beta-III spectrin mutations are a novel cause of neurodegenerative disease, which may affect the stabilization or trafficking of membrane proteins. PMID: 21827906
- TGF-beta signaling, particularly beta2SP, plays a critical role in hepatocyte proliferation and transitional phenotype. PMID: 20131405
- A mouse model lacking full-length beta-III spectrin reproduces features of human spinocerebellar ataxia type 5 including gait abnormalities, tremor, deteriorating motor coordination, Purkinje cell loss, and cerebellar atrophy. PMID: 20371805
- beta-III spectrin (SPTBN2) mutations cause spinocerebellar ataxia type 5 (SCA5) in an 11-generation American kindred descended from President Lincoln's grandparents and two additional families. PMID: 16429157
- None of the Spinocerebellar Ataxia individuals tested had evidence for one of the known SCA5 mutations. PMID: 17940722
- Adducin acting through spectrin provides a novel mechanism to regulate global properties of the lateral membrane of bronchial epithelial cells. PMID: 18003973
- the crystal structure of the ankyrin-binding domain of human beta2-spectrin at 1.95 A resolution together with mutagenesis data identifying the binding surface for ankyrins on beta2-spectrin. PMID: 19098307
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相關(guān)疾病：Spinocerebellar ataxia 5 (SCA5); Spinocerebellar ataxia, autosomal recessive, 14 (SCAR14)
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亞細(xì)胞定位：Cytoplasm, cytoskeleton. Cytoplasm, cell cortex.
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蛋白家族：Spectrin family
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組織特異性：Highly expressed in brain, kidney, pancreas, and liver, and at lower levels in lung and placenta.
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數(shù)據(jù)庫(kù)鏈接：
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