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Rabbit Anti-TBCE  antibody (bs-11767R)
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说明书: 50ul  100ul  200ul
50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包装/询价
产品编号 bs-11767R
英文名称 TBCE
中文名称 微管蛋白特定伴侣蛋白E抗体
别    名 HRD; KCS; KCS1; Pac2; tbce; TBCE_HUMAN; Tubulin specific chaperone e; Tubulin-folding cofactor E; Tubulin-specific chaperone E.  
研究领域 神经生物学  信号转导  生长因子和激素  细胞骨架  细胞外基质  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 (predicted: Human,Mouse,Rat,Cow,Horse,Rabbit,Sheep)
产品应用 ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 59kDa
细胞定位 细胞浆 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human TBCE: 442-527/527 
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 Microtubules, the primary component of the cytoskeletal network, are highly dynamic structures composed of Alpha/Beta Tubulin heterodimers. Biosynthesis of functional microtubules involve the participation of several chaperones, termed Tubulin folding cofactors A (TBCA), D (TBCD), E (TBCE) and C (TBCC), that act on folding intermediates downstream of the cytosolic chaperon, alternatively named TCP. TBCE (tubulin folding cofactor E), also known as HRD, KCS, KCS1 or pac2, is a 527 amino acid cytoplasmic protein containing one CAP-Gly domain and seven LRR (leucine-rich) repeats. TBCE is involved in the second step of the Tubulin folding pathway and is implicated in the maintenance of the neuronal microtubule network. TBCE associates with microtubules and proteasomes, and protects against misfolded protein stress. Mutations in the gene encoding TBCE are the cause of hypoparathyroidism-retardation-dysmorphism syndrome and Kenny-Caffey syndrome type 1.

Function:
Tubulin-folding protein; involved in the second step of the tubulin folding pathway. Seems to be implicated in the maintenance of the neuronal microtubule network. Involved in regulation of tubulin heterodimer dissociation.

Subunit:
Supercomplex made of cofactors A to E. Cofactors A and D function by capturing and stabilizing tubulin in a quasi-native conformation. Cofactor E binds to the cofactor D-tubulin complex; interaction with cofactor C then causes the release of tubulin polypeptides that are committed to the native state. Cofactors B and E can form a heterodimer which binds to alpha-tubulin and enhances their ability to dissociate tubulin heterodimers.

Subcellular Location:
Cytoplasm (By similarity). Cytoplasm, cytoskeleton (By similarity).

DISEASE:
Defects in TBCE are a cause of hypoparathyroidism-retardation-dysmorphism syndrome (HRD) [MIM:241410]; also known as hypoparathyroidism with short stature, mental retardation, and seizures or Sanjad-Sakati syndrome. HRD is an autosomal recessive disorder reported almost exclusively in Middle Eastern populations.
Defects in TBCE are the cause of Kenny-Caffey syndrome type 1 (KCS1) [MIM:244460]. KCS1 is similar to HRD with the additional features of osteosclerosis and recurrent bacterial infections.

Similarity:
Belongs to the TBCE family. Contains 1 CAP-Gly domain.
Contains 7 LRR (leucine-rich) repeats.
Contains 1 LRRCT domain.

SWISS:
Q15813

Gene ID:
6905

Database links:

Entrez Gene: 6905 Human

Entrez Gene: 70430 Mouse

Entrez Gene: 361255 Rat

Omim: 604934 Human

SwissProt: Q15813 Human

SwissProt: Q8CIV8 Mouse

SwissProt: Q5FVQ9 Rat

Unigene: 727621 Human

Unigene: 260209 Mouse

Unigene: 3547 Rat



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