Anti-SOD1/FITC Conjugated抗体上海钰博生物科技有限公司

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产品名称: Anti-SOD1/FITC Conjugated抗体
英文名称: Anti-SOD1/FITC
抗体货号: YB--1079R-FITC
产品规格: 20ul/50ul/100ul/200ul
级    别: , 分析纯, 见说明书
产品产地: 中国/美国
品牌商标: Ybscience
价    格: 2980元
抗原: KLH conjugated synthetic peptide derived from human SOD1 (101-154aa)
抗原来源: Rabbit
抗体来源: Rabbit
适用物种: Human, Mouse, Rat, Cow,
见说明书conjugate:见说明书
Isotype:见说明书
应用范围: 科研使用
更新时间: 2019/5/16 9:58:00
详细资料:  实验方法技术资料
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使用范围:仅限科研使用,不能应用于临床
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产品详细描述

 Anti-SOD1/FITC Conjugated抗体

产品编号 YB-1079R-FITC
英文名称 Anti-SOD1/FITC
中文名称 FITC标记的超氧化物歧化酶1/铜,锌过氧化物歧化酶SOD抗体
别    名 Superoxide Dismutase 1; ALS 1; ALS; ALS1; Amyotrophic lateral sclerosis 1 adult; Amyotrophic lateral sclerosis 1; Cu/Zn SOD; Cu/Zn superoxide dismutase; Homodimer; Indophenoloxidase A; IPOA; Mn superoxide dismutase; SOD 1; SOD; SOD soluble; SOD1; SOD2; SODC; Soluble indophenoloxidase A; Superoxide dismutase 1; Superoxide dismutase 1 soluble; Superoxide dismutase Cu Zn; Superoxide dismutase cystolic; SODC_HUMAN; Superoxide dismutase [Cu-Zn]; hSod1; Ipo1; SODC; Ipo-1; Sod-1; CuZnSOD; Cu/Zn-SOD; MGC107553; B430204E11Rik; superoxide-dimutase-1.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 神经生物学  细胞凋亡  激酶和***酸酶  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Cow, 
产品应用
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 17kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human SOD1 (101-154aa)
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
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.
产品介绍 background:
The protein encoded by this gene binds copper and zinc ions and is one of two isozymes responsible for destroying free superoxide radicals in the body. The encoded isozyme is a soluble cytoplasmic protein, acting as a homodimer to convert naturally-occuring but harmful superoxide radicals to molecular oxygen and hydrogen peroxide. The other isozyme is a mitochondrial protein. Mutations in this gene have been implicated as causes of familial amyotrophic lateral sclerosis. Rare transcript variants have been reported for this gene. [provided by RefSeq, Jul 2008]

Function:
Destroys radicals which are normally produced within the cells and which are toxic to biological systems.

Subunit:
Homodimer; non-disulfide linked. Homodimerization may take place via the ditryptophan cross-link at Trp-33. The pathogenic variants ALS1 Arg-38, Arg-47, Arg-86 and Ala-94 interact with RNF19A, whereas wild-type protein does not. The pathogenic variants ALS1 Arg-86 and Ala-94 interact with MARCH5, whereas wild-type protein does not.

Subcellular Location:
Cytoplasm. Note=The pathogenic variants ALS1 Arg-86 and Ala-94 gradually aggregates and accumulates in mitochondria.

Post-translational modifications:
Unlike wild-type protein, the pathogenic variants ALS1 Arg-38, Arg-47, Arg-86 and Ala-94 are polyubiquitinated by RNF19A leading to their proteasomal degradation. The pathogenic variants ALS1 Arg-86 and Ala-94 are ubiquitinated by MARCH5 leading to their proteasomal degradation. 
The ditryptophan cross-link at Trp-33 is responsible for the non-disulfide-linked homodimerization. Such modification might only occur in extreme conditions and additional experimental evidence is required.

DISEASE:
Defects in SOD1 are the cause of amyotrophic lateral sclerosis type 1 (ALS1) [MIM:105400]. ALS1 is a familial form of amyotrophic lateral sclerosis, a neurodegenerative disorder affecting upper and lower motor neurons and resulting in fatal paralysis. Sensory abnormalities are absent. Death usually occurs within 2 to 5 years. The etiology of amyotrophic lateral sclerosis is likely to be multifactorial, involving both genetic and environmental factors. The disease is inherited in 5-10% of cases leading to familial forms.

Similarity:
Belongs to the Cu-Zn superoxide dismutase family.

Database links:

Entrez Gene: 6647 Human

Entrez Gene: 20655 Mouse

Entrez Gene: 24786 Rat

Omim: 147450 Human

SwissProt: P00441 Human

SwissProt: P08228 Mouse

SwissProt: P07632 Rat

Unigene: 443914 Human

Unigene: 276325 Mouse

Unigene: 466779 Mouse

Unigene: 6059 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 

超氧化物歧化酶又称铜/锌过氧化物歧化酶SOD(Superoxide dismutase,简称SOD)是参与机体抗氧化(ROS,反应性氧离子reactive oxygen species)防御机制和抵御细胞氧化损伤最重要的酶类之一, 广泛存在于需氧生物、耐氧生物及某些厌氧微生物中,目前已知的SOD 主要分为三类,即胞质中Cu/Zn-SOD(即SOD1)、线粒体中的Mn-SOD(即SOD2)和ec-SOD(即SOD3)。
超氧化物歧化酶-1SOD1的水平与很多生理反应有关,如:应急,热休克,紫外和X线照射等。SOD1水平降低能触发AP2转录因子的激活。SOD1在临床上对很多疾病诊断有重要意义

生物在线声明:以上所展示的信息由企业自行提供,内容的真实性、准确性和合法性由发布企业负责。生物在线对此不承担任何保证责任。

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