• 阳性对照
  • Human, Mouse, Rat or Dogfish/Catshark Brain Tissue ICC/IF: SKNSH, Neuro-2A and NGF-differentiated PC12 cells IHC: Hu Cerebellum (FFPE tissue) Flow Cytometry: U-87MG cells, Neuro 2A cells.

性能

• 形式Liquid
• 存放说明Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.
• 存储溶液Preservative: 0.02% Sodium Azide
  Constituents: 1% BSA, PBS, pH 7.4
• 浓度Batch dependent within range: 100 µg 浓度为 0.5 - 1 mg/ml 
  

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• 纯度Immunogen affinity purified
• 克隆多克隆
• 同种型IgG
• 研究领域
  • Neuroscience
   
  • Cell Adhesion Proteins
   
  • Cytoskeletal Proteins
   
  • Microtubules

  • Signal Transduction
   
  • Cytoskeleton / ECM
   
  • Cytoskeleton
   
  • Microtubules
  • Tubulin

  • Tags & Cell Markers
   
  • Cell Type Markers
   
  • Neuroscience Markers
   
  • Neuronal

应用

靶标

• 功能Tubulin is the major constituent of microtubules. It binds two moles of GTP, one at an exchangeable site on the beta chain and one at a non-exchangeable site on the alpha-chain. TUBB3 plays a critical role in proper axon guidance and mantainance.
• 组织特异性Expression is primarily restricted to central and peripheral nervous system.
• 疾病相关Defects in TUBB3 are the cause of congenital fibrosis of extraocular muscles type 3A (CFEOM3A) [MIM:600638]. A congenital ocular motility disorder marked by restrictive ophthalmoplegia affecting extraocular muscles innervated by the oculomotor and/or trochlear nerves. It is clinically characterized by anchoring of the eyes in downward gaze, ptosis, and backward tilt of the head. Congenital fibrosis of extraocular muscles type 3 presents as a non-progressive, autosomal dominant disorder with variable expression. Patients may be bilaterally or unilaterally affected, and their oculo-motility defects range from complete ophthalmoplegia (with the eyes fixed in a hypo- and exotropic position), to mild asymptomatic restrictions of ocular movement. Ptosis, refractive error, amblyopia, and compensatory head positions are associated with the more severe forms of the disorder. In some cases the ocular phenotype is accompanied by additional features including developmental delay, corpus callosum agenesis, basal ganglia dysmorphism, facial weakness, polyneuropathy.
• 序列相似性Belongs to the tubulin family.
• 结构域The highly acidic C-terminal region may bind cations such as calcium.
• 翻译后修饰Some glutamate residues at the C-terminus are polyglutamylated. This modification occurs exclusively on glutamate residues and results in polyglutamate chains on the gamma-carboxyl group. Also monoglycylated but not polyglycylated due to the absence of functional TTLL10 in human. Monoglycylation is mainly limited to tubulin incorporated into axonemes (cilia and flagella) whereas glutamylation is prevalent in neuronal cells, centrioles, axonemes, and the mitotic spindle. Both modifications can coexist on the same protein on adjacent residues, and lowering glycylation levels increases polyglutamylation, and reciprocally. The precise function of such modifications is still unclear but they regulate the assembly and dynamics of axonemal microtubules.
• 细胞定位Cytoplasm > cytoskeleton.
• Information by UniProt
• 数据库链接
  • Entrez Gene: 10381 Human
  • Entrez Gene: 22152 Mouse
  • Entrez Gene: 246118 Rat
  • Omim: 602661 Human
  • SwissProt: Q13509 Human
  • SwissProt: Q9ERD7 Mouse