產(chǎn)品(pǐn)中(zhōng)心
標(biāo)記一抗
FITC標記(jì)的小腦脊髓共濟失調(tiáo)蛋(dàn)白(bái)3抗(kàng)體(tǐ)
英(yīng)文名(míng)稱Anti-ATXN3L/FITC
中文(wén)名稱(chēng):FITC標記(jì)的小(xiǎo)腦(nǎo)脊髓(suǐ)共濟(jì)失(shī)調蛋白(bái)3抗(kàng)體(tǐ)
別 名(míng)ATX3L_HUMAN; ATXN3L; Machado-Joseph disease protein 1-like; MJDL; Putative ataxin-3-like protein.
規(guī)格(gé):100ul
說(shuō) 明 書(shū)100ul
研究領(lǐng)域細胞生(shēng)物 免疫學(xué) 染色質和(hé)核(hé)信號(hào) 表(biǎo)觀(guān)遺傳(chuán)學(xué) 泛素(sù)
抗(kàng)體(tǐ)來(lái)源(yuán)Rabbit
克(kè)隆(lóng)類(lèi)型Polyclonal
交叉(chā)反(fǎn)應 Human,
產(chǎn)品應用(yòng)IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量41kDa
細(xì)胞(bāo)定位(wèi)細(xì)胞膜(mó)
性(xìng) 狀(zhuàng)Lyophilized or Liquid
濃 度1mg/ml
免 疫 原(yuán)KLH conjugated synthetic peptide derived from human ATXN3L
亞 型(xíng)IgG
純(chún)化方(fāng)法(fǎ)affinity purified by Protein A
儲(chǔ) 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存(cún)條件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.
產品(pǐn)介(jiè)紹(shào)background:
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.
Database links:
UniProtKB/Swiss-Prot: Q9H3M9.2
中文(wén)名稱(chēng):FITC標記(jì)的小(xiǎo)腦(nǎo)脊髓(suǐ)共濟(jì)失(shī)調蛋白(bái)3抗(kàng)體(tǐ)
別 名(míng)ATX3L_HUMAN; ATXN3L; Machado-Joseph disease protein 1-like; MJDL; Putative ataxin-3-like protein.
詳細介(jiè)紹：
規(guī)格(gé):100ul
說(shuō) 明 書(shū)100ul
研究領(lǐng)域細胞生(shēng)物 免疫學(xué) 染色質和(hé)核(hé)信號(hào) 表(biǎo)觀(guān)遺傳(chuán)學(xué) 泛素(sù)
抗(kàng)體(tǐ)來(lái)源(yuán)Rabbit
克(kè)隆(lóng)類(lèi)型Polyclonal
交叉(chā)反(fǎn)應 Human,
產(chǎn)品應用(yòng)IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量41kDa
細(xì)胞(bāo)定位(wèi)細(xì)胞膜(mó)
性(xìng) 狀(zhuàng)Lyophilized or Liquid
濃 度1mg/ml
免 疫 原(yuán)KLH conjugated synthetic peptide derived from human ATXN3L
亞 型(xíng)IgG
純(chún)化方(fāng)法(fǎ)affinity purified by Protein A
儲(chǔ) 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存(cún)條件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.
相(xiāng)關(guān)資(zī)料(liào)：
產品(pǐn)介(jiè)紹(shào)background:
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.
Database links:
UniProtKB/Swiss-Prot: Q9H3M9.2
