Rabbit Anti-TBCE/FITC Conjugated antibody
|别 名||HRD; KCS; KCS1; Pac2; tbce; TBCE_HUMAN; Tubulin specific chaperone e; Tubulin-folding cofactor E; Tubulin-specific chaperone E.|
|规格价格||100ul/2980元 购买 大包装/询价|
|说 明 书||100ul|
|研究领域||神经生物学 信号转导 生长因子和*** 细胞骨架 细胞外基质|
|交叉反应||Human, Mouse, Rat, Dog, Cow, Rabbit,|
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
|分 子 量||59kDa|
|性 状||Lyophilized or Liquid|
|免 疫 原||KLH conjugated synthetic peptide derived from human TBCE|
|纯化方法||affinity purified by Protein A|
|储 存 液||0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.|
|保存条件||Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.|
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.
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.
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.
Cytoplasm (By similarity). Cytoplasm, cytoskeleton (By similarity).
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.
Belongs to the TBCE family. Contains 1 CAP-Gly domain.
Contains 7 LRR (leucine-rich) repeats.
Contains 1 LRRCT domain.
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
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic application
微管是细胞骨架网络的主要组成部分，是由Alpha/Beta管蛋白异源二聚体组成的高度动态的结构。功能微管的生物合成涉及几个伴侣的参与，称为管蛋白折叠辅因子A(TBCA)、D(TBCD)、E(TBCE)和C(TBCC)，它们作用于细胞质伴侣下游的折叠中间体，或者称为TCP。TBCE(tubulin folding cofactor E)又称HRD、KCS、KCS1或pac2，是含有一个CAP-Gly结构域和7个LRR(富含亮氨酸)重复序列的527个氨基酸胞浆蛋白。TBCE参与小管蛋白折叠途径的第二步，并参与维持神经元微管网络。TBCE与微管和蛋白酶体结合，并防止错误折叠的蛋白压力。TBCE基因突变是甲状旁腺功能减退-发育迟缓-畸形综合征和肯尼-卡菲综合征1型的病因。