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小腦脊髓共濟失調(diào)蛋白3抗體
  • 產(chǎn)品貨號：
    BN41018R
  • 中文名稱：
    小腦脊髓共濟失調(diào)蛋白3抗體
  • 英文名稱：
    Rabbit anti-ATXN3L Polyclonal antibody
  • 品牌：
    Biorigin

  • 貨號

    產(chǎn)品規(guī)格

    售價

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  • BN41018R-100ul

    100ul

    ￥2360.00

    交叉反應(yīng)：Human 推薦應(yīng)用：WB,IHC-P,IHC-F,IF

  • BN41018R-200ul

    200ul

    ￥3490.00

    交叉反應(yīng)：Human 推薦應(yīng)用：WB,IHC-P,IHC-F,IF

產(chǎn)品描述

英文名稱ATXN3L
中文名稱小腦脊髓共濟失調(diào)蛋白3抗體
別    名ATX3L_HUMAN; ATXN3L; Machado-Joseph disease protein 1-like; MJDL; Putative ataxin-3-like protein.  
研究領(lǐng)域細(xì)胞生物  免疫學(xué)  染色質(zhì)和核信號  表觀遺傳學(xué)  泛素  
抗體來源Rabbit
克隆類型Polyclonal
交叉反應(yīng)Human, 
產(chǎn)品應(yīng)用WB=1:500-2000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:50-200 （石蠟切片需做抗原修復(fù)）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量41kDa
細(xì)胞定位細(xì)胞膜 
性    狀Liquid
濃    度1mg/ml
免 疫 原KLH conjugated synthetic peptide derived from human ATXN3L:251-355/355 
亞    型IgG
純化方法affinity purified by Protein A
儲 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
PubMedPubMed
產(chǎn)品介紹Defects in ATXN3 are the cause of spinocerebellar ataxia type 3 (SCA3) ; also known as Machado-Joseph disease (MJD). Spinocerebellar ataxia is a clinically and genetically heterogeneous group of cerebellar disorders. Patients show progressive incoordination of gait and often poor coordination of hands, speech and eye movements, due to degeneration of the cerebellum with variable involvement of the brainstem and spinal cord. SCA3 belongs to the autosomal dominant cerebellar ataxias type I (ADCA I) which are characterized by cerebellar ataxia in combination with additional clinical features like optic atrophy, ophthalmoplegia, bulbar and extrapyramidal signs, peripheral neuropathy and dementia. The molecular defect in SCA3 is the a CAG repeat expansion in ATXN3 coding region. Longer expansions result in earlier onset and more severe clinical manifestations of the disease.

Function:
Deubiquitinating enzyme that cleaves both 'Lys-48'-linked and 'Lys-63'-linked poly-ubiquitin chains (in vitro).

Subcellular Location:
Nucleus (By similarity).

Similarity:
Contains 1 Josephin domain.
Contains 2 UIM (ubiquitin-interacting motif) repeats.

SWISS:
Q9H3M9

Gene ID:
25814

Database links:

Entrez Gene: 25814 Human

Entrez Gene: 170821 Rat

Omim: 611150 Human

SwissProt: Q9UBB4 Human

SwissProt: Q9ER24 Rat

Unigene: 475125 Human

Unigene: 6524 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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