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    Rabbit Anti-TBCE/Gold Conjugated antibody (bs-20328R-Gold)
    訂購熱線(xiàn):400-901-9800
    訂購郵箱:sales@bioss.com.cn
    訂購QQ:  400-901-9800
    技術(shù)支持:techsupport@bioss.com.cn
    說(shuō) 明 書(shū): 100ul(10nm  15nm  35nm
    100ul/2980.00元
    大包裝/詢(xún)價(jià)
    產(chǎn)品編號 bs-20328R-Gold
    英文名稱(chēng) Rabbit Anti-TBCE/Gold Conjugated antibody
    中文名稱(chēng) 膠體金標記的微管蛋白特定伴侶蛋白E抗體
    別    名 HRD; KCS; KCS1; Pac2; tbce; TBCE_HUMAN; Tubulin specific chaperone e; Tubulin-folding cofactor E; Tubulin-specific chaperone E.  
    規格價(jià)格 100ul/2980元 購買(mǎi)        大包裝/詢(xún)價(jià)
    說(shuō) 明 書(shū) 100ul(10nm  15nm  35nm
    研究領(lǐng)域 神經(jīng)生物學(xué)  信號轉導  生長(cháng)因子和激素  細胞骨架  細胞外基質(zhì)  
    抗體來(lái)源 Rabbit
    克隆類(lèi)型 Polyclonal
    交叉反應 (predicted: Human, Mouse, Rat, Dog, Cow, Rabbit, )
    產(chǎn)品應用 IEM=1:20-200 ICA=1:20-200 ChIP=1:20-200 
    not yet tested in other applications.
    optimal dilutions/concentrations should be determined by the end user.
    分 子 量 59kDa
    性    狀 Lyophilized or Liquid
    濃    度 0.4mg/ml
    免 疫 原 KLH conjugated synthetic peptide derived from human TBCE
    亞    型 IgG
    純化方法 affinity purified by Protein A
    儲 存 液 0.02M TBS(pH8.2) with 1% BSA, 0.03% Proclin300.
    保存條件 Store at 2-8 oC for 3-6 months. Avoid repeated freeze/thaw cycles.
    產(chǎn)品介紹 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 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.


    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.
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