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英文名稱(chēng)Anti-ATX2/FITC
中文名稱(chēng):FITC標(biāo)記的脊髓小腦共濟(jì)失調(diào)2型蛋白抗體
別 名Ataxin 2; ATXN2; Olivopontocerebellar ataxia 2, autosomal dominant; SCA2; Spinocerebellar ataxia type 2 protein; TNRC13; Trinucleotide repeat containing gene 13 protein; SRRT_HUMAN.
規(guī)格:100ul
說(shuō) 明 書(shū)100ul
研究領(lǐng)域細(xì)胞生物 免疫學(xué) 神經(jīng)生物學(xué) 表觀(guān)遺傳學(xué)
抗體來(lái)源Rabbit
克隆類(lèi)型Polyclonal
交叉反應(yīng) Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse,
產(chǎn)品應(yīng)用IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量101kDa
性 狀Lyophilized or Liquid
濃 度1mg/ml
免 疫 原KLH conjugated synthetic peptide derived from human ATX2 (771-815aa)
亞 型IgG
純化方法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(chǎn)品介紹background:
The autosomal dominant cerebellar ataxias (ADCA) are a heterogeneous group of neurodegenerative disorders characterized by progressive degeneration of the cerebellum, brain stem and spinal cord. Clinically, ADCA has been divided into three groups: ADCA types I-III. ATX2 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. ATX2 is caused by expansion of a CAG repeat in the coding region of ATX2. Longer expansions result in earlier onset of the disease. There are four named isoforms.
Subunit:
Monomer. Can also form homodimers.
Subcellular Location:
Cytoplasm.
Tissue Specificity:
Expressed in the brain, heart, liver, skeletal muscle, pancreas and placenta. Isoform 1 is predominant in the brain and spinal cord. Isoform 4 is more abundant in the cerebellum. In the brain, broadly expressed in the amygdala, caudate nucleus, corpus callosum, hippocampus, hypothalamus, substantia nigra, subthalamic nucleus and thalamus.
Similarity:
Belongs to the ataxin-2 family.
中文名稱(chēng):FITC標(biāo)記的脊髓小腦共濟(jì)失調(diào)2型蛋白抗體
別 名Ataxin 2; ATXN2; Olivopontocerebellar ataxia 2, autosomal dominant; SCA2; Spinocerebellar ataxia type 2 protein; TNRC13; Trinucleotide repeat containing gene 13 protein; SRRT_HUMAN.
詳細(xì)介紹：
規(guī)格:100ul
說(shuō) 明 書(shū)100ul
研究領(lǐng)域細(xì)胞生物 免疫學(xué) 神經(jīng)生物學(xué) 表觀(guān)遺傳學(xué)
抗體來(lái)源Rabbit
克隆類(lèi)型Polyclonal
交叉反應(yīng) Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse,
產(chǎn)品應(yīng)用IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量101kDa
性 狀Lyophilized or Liquid
濃 度1mg/ml
免 疫 原KLH conjugated synthetic peptide derived from human ATX2 (771-815aa)
亞 型IgG
純化方法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.
相關(guān)資料：
產(chǎn)品介紹background:
The autosomal dominant cerebellar ataxias (ADCA) are a heterogeneous group of neurodegenerative disorders characterized by progressive degeneration of the cerebellum, brain stem and spinal cord. Clinically, ADCA has been divided into three groups: ADCA types I-III. ATX2 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. ATX2 is caused by expansion of a CAG repeat in the coding region of ATX2. Longer expansions result in earlier onset of the disease. There are four named isoforms.
Subunit:
Monomer. Can also form homodimers.
Subcellular Location:
Cytoplasm.
Tissue Specificity:
Expressed in the brain, heart, liver, skeletal muscle, pancreas and placenta. Isoform 1 is predominant in the brain and spinal cord. Isoform 4 is more abundant in the cerebellum. In the brain, broadly expressed in the amygdala, caudate nucleus, corpus callosum, hippocampus, hypothalamus, substantia nigra, subthalamic nucleus and thalamus.
Similarity:
Belongs to the ataxin-2 family.
