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TUBB3, BF680 conjugated (bs-4512R-BF680)  
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100ul/2980.00元
大包装/询价
产品编号 bs-4512R-BF680
英文名称 TUBB3, BF680 conjugated
中文名称 BF680标记的神经细胞特异性微管蛋白抗体
别    名 Neuron specific beta III Tubulin; beta 4; MC1R; TBB3_HUMAN; TUBB 3; TUBB 4; TUBB3; TUBB4; Tubulin beta 3 chain; Tubulin beta 4; Tubulin beta III; Tubulin beta-3 chain; Tubulin beta-4 chain; Tubulin beta-III; Beta tubulin III; Neuron specific beta III Tubulin.  
研究领域 细胞生物  免疫学  神经生物学  转录调节因子  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应
产品应用
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 50-55kDa
细胞定位 细胞浆 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human beta III Tubulin: 401-450/450 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
产品介绍 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.

Function:
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.

Subcellular Location:
Cytoplasm, cytoskeleton.

Tissue Specificity:
Expression is primarily restricted to central and peripheral nervous system.

Post-translational modifications:
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.

DISEASE:
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.

Similarity:
Belongs to the tubulin family.

SWISS:
Q13509

Gene ID:
10381

Database links:

Entrez Gene: 10381 Human

Entrez Gene: 431043 Chicken

Entrez Gene: 22152 Mouse

Entrez Gene: 246118 Rat

Omim: 602661 Human

SwissProt: Q2T9S0 Cow

SwissProt: Q13509 Human

SwissProt: Q9ERD7 Mouse

SwissProt: Q4QRB4 Rat

Unigene: 511743 Human

Unigene: 40068 Mouse

Unigene: 43958 Rat



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