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英文名稱Anti-Ataxin 7/FITC
中文名稱:FITC標記的脊髓小腦共濟失調蛋白7抗體
別 名Ataxin7; Ataxin-7; ADCAII; ATXN 7; OPCA III; OPCA3; SCA 7; SCA7; Spinocerebellar Ataxia 7; Spinocerebellar ataxia type 7 protein; ATX7_HUMAN.
規格:100ul
說 明 書100ul
研究領域細胞生物 神經生物學
抗體來源Rabbit
克隆類型Polyclonal
交叉反應 Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep,
產品應用ICC=1:50-200 IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量95kDa
性 狀Lyophilized or Liquid
濃 度1mg/ml
免 疫 原KLH conjugated synthetic peptide derived from human Ataxin 7
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.
產品介紹background:
The human ataxin-7 gene, also known as spinocerebellar ataxia 7 or SCA7, maps to chromosome 3p13-p12, has a 2,727-bp open reading frame, and encodes a 892 amino acid protein containing a nuclear localization signal and a polyglutamine tract (1,2). SCA7 is an autosomal dominant neurodegenerative disorder characterized by ataxia and selective neuronal cell loss caused by the expansion of a translated CAG repeat encoding a polyglutamine tract in ataxin-7, which is the SCA7 gene product (3,4). Ataxin-7 is expressed within neurons both affected and unaffected in SCA7 pathology with subcellular localization being variable depending upon the neuronal subtype (5). Polyglutamine expanded in ataxin-7 may carry out its pathogenic effects in the nucleus by altering the matrix-associated nuclear structure and/or by disrupting nucleolar function (6)
Function:
Ataxin 7 is a protein of unknown function. It may be the human orthologue of the yeast SAGA SGF73 subunit and a subunit of the human TFTC-like transcriptional complexes. Spinocerebellar ataxia 7 (one of a group of hereditary neurodegenrative diseases) is caused by an expanded trinucleotide repeat in the gene encoding ataxin 7. Ataxin 7 is typically located in the cytoplasm and on the nuclear membrane of normal brain neurons. In cells where there is a mutation of the SCA7 gene, ataxin 7 accumulates in intranuclear inclusions and can result in cell death.
Subunit:
Component of the STAGA transcription coactivator-HAT complex, at least composed of SUPT3H, GCN5L2, TAF5L, TAF6L, SUPT7L, TADA3L, TAD1L, TAF10, TAF12, TRRAP, TAF9 and ATXN7. The STAGA core complex is associated with a subcomplex required for histone deubiquitination composed of ATXN7L3, ENY2 and USP22. Interacts with SORBS1, PSMC1 and CRX. Interacts with TRRAP, GCN5L2 and TAF10. Interacts with alpha tubulin.
Subcellular Location:
Cytoplasmic (isoform b) and Nuclear (isoform a)
Tissue Specificity:
Isoform a and isoform b are expressed in CNS, but isoform a is expressed predominantly in the peripherical tissues. Isoform b is also highly expressed in the frontal lobe, skeletal muscle and spinal cord and is expressed at a lower level in the lung, lymphoblast and intestine.
Post-translational modifications:
Proteolytically cleaved. The cleavage may be involved in SCA7 degeneration: the isoform fragments may exert distinct toxic influences that could contribute to selective neurodegeneration.
Sumoylation decreases the aggregation propensity and cellular toxicity of forms with an expanded poly-Gln region but has no effect on subcellular location or interaction with components of the STAGA complex.
中文名稱:FITC標記的脊髓小腦共濟失調蛋白7抗體
別 名Ataxin7; Ataxin-7; ADCAII; ATXN 7; OPCA III; OPCA3; SCA 7; SCA7; Spinocerebellar Ataxia 7; Spinocerebellar ataxia type 7 protein; ATX7_HUMAN.
詳細介紹：
規格:100ul
說 明 書100ul
研究領域細胞生物 神經生物學
抗體來源Rabbit
克隆類型Polyclonal
交叉反應 Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep,
產品應用ICC=1:50-200 IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量95kDa
性 狀Lyophilized or Liquid
濃 度1mg/ml
免 疫 原KLH conjugated synthetic peptide derived from human Ataxin 7
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.
相關資料：
產品介紹background:
The human ataxin-7 gene, also known as spinocerebellar ataxia 7 or SCA7, maps to chromosome 3p13-p12, has a 2,727-bp open reading frame, and encodes a 892 amino acid protein containing a nuclear localization signal and a polyglutamine tract (1,2). SCA7 is an autosomal dominant neurodegenerative disorder characterized by ataxia and selective neuronal cell loss caused by the expansion of a translated CAG repeat encoding a polyglutamine tract in ataxin-7, which is the SCA7 gene product (3,4). Ataxin-7 is expressed within neurons both affected and unaffected in SCA7 pathology with subcellular localization being variable depending upon the neuronal subtype (5). Polyglutamine expanded in ataxin-7 may carry out its pathogenic effects in the nucleus by altering the matrix-associated nuclear structure and/or by disrupting nucleolar function (6)
Function:
Ataxin 7 is a protein of unknown function. It may be the human orthologue of the yeast SAGA SGF73 subunit and a subunit of the human TFTC-like transcriptional complexes. Spinocerebellar ataxia 7 (one of a group of hereditary neurodegenrative diseases) is caused by an expanded trinucleotide repeat in the gene encoding ataxin 7. Ataxin 7 is typically located in the cytoplasm and on the nuclear membrane of normal brain neurons. In cells where there is a mutation of the SCA7 gene, ataxin 7 accumulates in intranuclear inclusions and can result in cell death.
Subunit:
Component of the STAGA transcription coactivator-HAT complex, at least composed of SUPT3H, GCN5L2, TAF5L, TAF6L, SUPT7L, TADA3L, TAD1L, TAF10, TAF12, TRRAP, TAF9 and ATXN7. The STAGA core complex is associated with a subcomplex required for histone deubiquitination composed of ATXN7L3, ENY2 and USP22. Interacts with SORBS1, PSMC1 and CRX. Interacts with TRRAP, GCN5L2 and TAF10. Interacts with alpha tubulin.
Subcellular Location:
Cytoplasmic (isoform b) and Nuclear (isoform a)
Tissue Specificity:
Isoform a and isoform b are expressed in CNS, but isoform a is expressed predominantly in the peripherical tissues. Isoform b is also highly expressed in the frontal lobe, skeletal muscle and spinal cord and is expressed at a lower level in the lung, lymphoblast and intestine.
Post-translational modifications:
Proteolytically cleaved. The cleavage may be involved in SCA7 degeneration: the isoform fragments may exert distinct toxic influences that could contribute to selective neurodegeneration.
Sumoylation decreases the aggregation propensity and cellular toxicity of forms with an expanded poly-Gln region but has no effect on subcellular location or interaction with components of the STAGA complex.
