共济失调蛋白8抗体价格价格,详情介绍-960化工网

共济失调蛋白8抗体价格

价格：¥1000 - 3500

品牌：上海博湖

产地：进口、国产

最新更新日期：2021-08-12 08:36

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公司：上海博湖生物科技有限公司

联系人：韩丽君

联系电话： 18117197628 021-57763112

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产品属性

抗体名	共济失调蛋白8抗体价格

抗体英文名

Anti-Twinkle/ATXN8

靶点

详见说明书

浓度

1mg/1ml

应用范围	WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500

宿主

详见说明书

供应商

上海博湖

数量

大量

级别

详见说明书

目录编号

详见说明书

抗原来源

Rabbit

保质期

详见说明书

适应物种

详见说明书

标记物

详见说明书

克隆性

多克隆

保存条件	Store at -20 °C

形态

详见说明书

亚型

IgG

免疫原	KLH conjugated synthetic peptide derived from hu Twinkle/ATXN8

规格	0.2ml/200μg

产品说明

共济失调蛋白8抗体价格是用于化学反应、分析化验、研究实验、教学实验、化学配方使用的纯净化学品，产品品质卓越，价格实惠，多种规格供应，售后完善。
英文名称 Anti-Twinkle/ATXN8
中文名称共济失调蛋白8抗体价格
别名 Ataxin 8; Ataxin8; ATXN 8; ATXN8; C10 orf2; C10orf 2; C10orf2; Chromosome 10 open reading frame 2; IOSCA; PEO 1; PEO; PEO1; PEOA3; Progressive external ophthalmoplegia 1 protein; SANDO; SCA 8; SCA8; T7 gp4 like protein with intramitochondrial nucleoid localization; T7 like mitochondrial DNA helicase; Twinkle protein, mitochondrial; TWINL.

浓度 1mg/1ml
规格 0.2ml/200μg
抗体来源 Rabbit
克隆类型 polyclonal
交叉反应 Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit
产品类型一抗
研究领域细胞生物神经生物学线粒体表观遗传学
蛋白分子量 predicted molecular weight: 77kDa
性状 Lyophilized or Liquid
免疫原 KLH conjugated synthetic peptide derived from hu Twinkle/ATXN8
亚型 IgG
纯化方法 affinity purified by Protein A
储存液 Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4

共济失调蛋白8抗体价格产品应用 WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500
（石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
保存条件 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.
Important Note This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
产品介绍 Twinkle, also known as PEO1 (Progressive external ophthalmoplegia 1 protein), PEOA3, SANDO or TWINL, is a mitochondrial protein that functions as a 5’-3’ nucleotide-dependent DNA helicase. Colocalized with mtDNA (mitochondrial DNA) in mitochondrial nucleoids, Twinkle is important in the metabolism and maintenance of mtDNA, playing a crucial role in the regulation of mtDNA copy numbers. Twinkle is expressed at high levels in testis, pancreas and skeletal muscle and exists as three isoforms due to alternative splicing events. Defects in the gene encoding Twinkle are the cause of two conditions: progressive external ophthalmoplegia with mitochondrial DNA deletions autosomal dominant 3 (PEOA3) and sensory ataxic neuropathy dysarthria and ophthalmoparesis (SANDO). PEOA3 is characterized by ptosis and weak muscles, while SANDO is characterized by ophthalmoparesis, dysarthria and sensory ataxic neuropathies.
Function : Twinkle, also known as PEO1, is involved in mitochondrial DNA (mtDNA) metabolism, possibly by functioning as an adenine nucleotide-dependent DNA helicase at the mitochondrial DNA replication fork. It may be a key regulator of mitochondrial DNA copy number in mammals. Mutations in Twinkle lead to infantile-onset spinocerebellar ataxia, a severe neurodegenerative disorder. Mutations in Twinkle are also associated with a number of diseases that manifest with symptoms such as sensory ataxia, neuropathy, ophthalmoplegia, and hearing loss.
Subunit : Forms multimers in vitro, including hexamers. Interacts with POLG in vitro.
Subcellular Location : Mitochondrion matrix, mitochondrion nucleoid. Note=Colocalizes with mtDNA in mitochondrial nucleoids, a nucleoproteins complex consisting of a number of copies of proteins associated with mtDNA, probably involved in mtDNA maintenance and expression.
Tissue Specificity : High relative levels in skeletal muscle, testis and pancreas. Lower levels of expression in the heart, brain, placenta, lung, liver, kidney, spleen, thymus, prostate, ovary, small intestine, colon and leukocytes. Expression is coregulated with MRPL43.
DISEASE : Progressive external ophthalmoplegia with mitochondrial DNA deletions autosomal dominant 3 (PEOA3) [MIM:609286]: A disorder characterized by progressive weakness of ocular muscles and levator muscle of the upper eyelid. In a minority of cases, it is associated with skeletal myopathy, which predominantly involves axial or proximal muscles and which causes abnormal fatigability and even permanent muscle weakness. Ragged-red fibers and atrophy are found on muscle biopsy. A large proportion of chronic ophthalmoplegias are associated with other symptoms, leading to a multisystemic pattern of this disease. Additional symptoms are variable, and may include cataracts, hearing loss, sensory axonal neuropathy, ataxia, depression, hypogonadism, and parkinsonism. Note=The disease is caused by mutations affecting the gene represented in this entry. [DISEASE] Sensory ataxic neuropathy dysarthria and ophthalmoparesis (SANDO) [MIM:607459]: A systemic disorder resulting from mitochondrial dysfunction associated with mitochondrial depletion in skeletal muscle and peripheral nerve tissue. The clinical triad of symptoms consists of sensory ataxic neuropathy, dysarthria, and ophthalmoparesis. However, the phenotype varies widely, even within the same family, and can also include myopathy, seizures, and hearing loss. An atypical form of the disease is characterized by headaches and/or seizures manifesting in childhood or adolescence, followed by development of cerebellar and sensory ataxia, dysarthria, progressive external ophthalmoplegia, and myoclonus in early adulthood. Note=The disease is caused by mutations affecting the gene represented in this entry.
Mitochondrial DNA depletion syndrome 7 (MTDPS7) [MIM:271245]: A severe disease associated with mitochondrial dysfunction. Some patients are affected by progressive atrophy of the cerebellum, brain stem, the spinal cord, and sensory axonal neuropathy. Clinical features include hypotonia, athetosis, ataxia, ophthalmoplegia, sensorineural hearing deficit, sensory axonal neuropathy, epileptic encephalopathy and female hypogonadism. In some individuals liver dysfunction and multi-organ failure is present. Note=The disease is caused by mutations affecting the gene represented in this entry.
Similarity : Contains 1 SF4 helicase domain.
Database links : UniProtKB/Swiss-Prot: Q96RR1.1

共济失调蛋白8抗体价格具有全、新、优、品、好四大特点：
全：公司提供上万种产品，涵盖了生物试剂，elisa试剂盒，标准品，培养基，原装耗材，抗体、培养基、ATCC细胞等，基本上各种科研所需产品在我司都能找到。
新：产品更新速度较快，基本上每周都有新产品出现。
优：产品质量好，投诉比较少。
好：我公司具有优质的技术团队，产品一旦售出，实验过程中遇到困难可提供在线技术咨询。使您使用产品时没有任何的后顾之忧。
我们提供的标记服务，可根据您科研的需求，满足您的需要，质量保证，价格合理。
一、标记流程
委托标记服务，请下载并详细填写《委托标记申请单》，待我公司标记室技术人员研究、确认后答复，并办理委托标记事宜。
二、各种标记物制备的收费标准（RMB）
我们不但提供抗体和大分子蛋白的标记服务，还提供小分子多肽及小分子蛋白的各种标记服务，提供标记化合物的服务（此化合物必须适用于标记），价格另议。
一抗和二坑的区别：
第一抗体就是平常所说的抗体，即能和抗原特异性结合。
第二抗体是能和抗体结合的，即抗体的抗体。主要用于检测抗体的存在。
一抗是针对抗原的抗体，二抗是针对一抗的抗体。即抗体也可以充当抗原刺激机体产生抗体。也就是说，抗原进入机体刺激机体免疫系统产生免疫应答，由B细胞可以产生与相应抗原发生特异性结合的特殊蛋白质。
一抗二抗都是一种可以特异结合别的物质的基团，而且一抗可以至少结合两种其他基团（底物和二抗）。
一抗：可以特异结合底物，就是识别出我们想要检测的东西。一抗和底物结合与否用肉眼是看不出来的。
二抗：可以和一抗结合，并带有可以被检测出的标记(如带荧光、放射性、化学发光或显色基团），作用是检测一抗。如果一抗自己带有可以被检测出的标记(如带荧光、放射性、化学发光或显色基团），则不需要二抗。但这样成本很高，因为一种一抗只识别一种底物。所以如今的设计一般是二抗带上可检测标记，再来检测一抗。而一抗识别底物。这样，当一抗结合到底物上，就可以通过二抗检测出来。

Anti-Phospho-eNOS (Thr495)/FITC 荧光素标记化合成酶3抗体(内皮型)IgGMulti-class antibodies规格： 0.2ml
Anti-ZO-1/FITC 荧光素标记胞质紧密粘连蛋白1抗体IgGMulti-class antibodies规格： 0.2ml
半胱酸蛋白酶8抗体 Anti-Caspase-8 0.1ml
Goat Anti-Guinea pig IgG/PE-CY5 PE-CY5标记的羊抗豚鼠IgG 0.1ml
FBXW8 英文名称： FBXW8蛋白抗体 0.2ml
Rhesus antibody Rh Pol II/RNA polymerase II RNA聚合酶II抗体规格 0.2ml
Anti-ZO-1/FITC 荧光素标记胞质紧密粘连蛋白1抗体IgGMulti-class antibodies规格： 0.2ml
MCP-4/CCL13(Human monocyte chemotactic protein 4) ELISA kit 人单核细胞趋化蛋白4Multi-class antibodies规格： 48T
Anti-FSH 促卵泡刺激素抗体Multi-class antibodies规格： 0.2ml
Rhesus antibody Rh Mouse Anti-human IgM/Alexa Fluor 647 Alexa Fluor 647标记的小鼠抗人IgM 规格 0.1ml
fMet(Human formylmine) ELISA Kit 人甲酰甲 96T
SLITRK4 英文名称：神经突触相关蛋白SLITRK4抗体 0.2ml
Collagen II 英文名称：Ⅱ型胶原α1蛋白/软骨素抗体 0.1ml
Anti-FSH 促卵泡刺激素抗体Multi-class antibodies规格： 0.2ml
Goat Anti-Guinea pig IgG/Cy5.5 Cy5.5标记的羊抗豚鼠IgG 0.1ml
FAM123B 英文名称：肾母细胞瘤X蛋白抗体 0.2ml
成骨特异性转录因子抗体 Anti-CBFA1/RUNX2 0.1ml
Anti-Notch3/FITC 荧光素标记跨膜受体蛋白Notch-3抗体IgGMulti-class antibodies规格： 0.2ml
Rhesus antibody Rh POC5 细胞中心粒蛋白抗体规格 0.2ml
Anti-TORC1/CRTC1/FITC 荧光素标记环腺苷酸应答元件结合蛋白转录共激活因子TORC1抗体IgGMulti-class antibodies规格： 0.2ml
CXCL3 Protein Human 重组人 CXCL3 / GRO gamma 蛋白 (His 标签)
人支气管成纤维细胞完全培养基 100mL
VEGFC Others Rat 大鼠 VEGFC / VEGF-C (aa 108-223) 人细胞裂解液 (阳性对照)
Y79细胞，人视网膜母细胞瘤球毛壳霉小鼠肺腺癌细胞系;LA795
盘羊皮肤细胞;ASHS2
Lncap clone FGC(人前列腺癌细胞) 5×106cells/瓶×2 人 AMTN / Amelotin 人细胞裂解液 (阳性对照)
大耳山羊肺细胞;LDG-1
IL7R Others Rat 大鼠 IL7R / IL7RA 人细胞裂解液 (阳性对照)
人呼吸道上皮细胞完全培养基 100mL
U2OS-Luc teT-on细胞，荧光素酶转染人成骨肉瘤细胞枯草芽孢杆菌小鼠肾集合管细胞(SV转化);M-1
CCL3 Protein Human 重组人 CCL3 / Mip1a 蛋白 (His 标签)
LoVo(人大肠癌细胞) 5×106cells/瓶×2 人 EDNRB / Endothelin B Receptor 人细胞裂解液 (阳性对照)
共济失调蛋白8抗体价格CXCL3 Protein Human 重组人 CXCL3 / GRO gamma 蛋白 (His 标签)
人支气管成纤维细胞完全培养基 100mL
VEGFC Others Rat 大鼠 VEGFC / VEGF-C (aa 108-223) 人细胞裂解液 (阳性对照)
Y79细胞，人视网膜母细胞瘤球毛壳霉小鼠肺腺癌细胞系;LA795
盘羊皮肤细胞;ASHS2
Lncap clone FGC(人前列腺癌细胞) 5×106cells/瓶×2 人 AMTN / Amelotin 人细胞裂解液 (阳性对照)