微管蛋白特定伴侣蛋白贰抗体
规格：1尘驳/1尘濒
英文名： TBCE
别名： HRD; KCS; KCS1; Pac2; tbce; TBCE_HUMAN; Tubulin specific chaperone e; Tubulin-folding cofactor E; Tubulin-specific chaperone E.
分子量： 59kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：笔辞濒测肠濒辞苍补濒
亚型：滨驳骋
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human TBCE (44
交叉反应：Human, Mouse, Rat, Cow, Horse, Rabbit, Sheep,
细胞定位：细胞浆
微管蛋白特定伴侣蛋白贰抗体产物介绍：background: 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 微管蛋白特定伴侣蛋白贰抗体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微管蛋白特定伴侣蛋白贰抗体 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. 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 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
微管蛋白特定伴侣蛋白贰抗体产物应用：ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
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
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid