货号 |
bs-1079R-1 |
品牌 |
|
浓度 |
|
货期 |
现货 |
英文名称 |
Rabbit Anti-SOD1 antibody |
中文名称 |
Rabbit Anti-SOD1 antibody |
研究领域 |
神经生物学,细胞凋亡,激酶和磷酸酶 |
英文别名 |
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;SOD2; SODC; Sol |
反应物种(已验证) |
Human,Mouse,Rat |
反应物种(预测) |
Cow |
产品应用(已验证) |
WB,IHC,ICC,FCM |
推荐稀释比例 |
WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,Flow Cyt=3ug/Test,ICC=1:100, |
克隆类型 |
多克隆 |
抗体来源 |
Rabbit |
理论分子量 |
17 |
细胞定位 |
细胞核,细胞浆 |
性状 |
Liquid |
免疫原 |
KLH conjugated synthetic peptide derived from human SOD1 |
抗原表位 |
101-154/154 |
亚型 |
IgG |
纯化方法 |
affinity purified by Protein A |
SUBCELLULAR |
Cytoplasm. Note=The pathogenic variants ALS1 Arg-86 and Ala-94 gradually aggregates and accumulates in mitochondria. |
SIMILARITY |
Belongs to the Cu-Zn superoxide dismutase family. |
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 AL |
Function |
Destroys radicals which are normally produced within the cells and which are toxic to biological systems. |
Post-translational |
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 |
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. Sen |
SWISS |
P00441 |
Gene ID |
6647 |
保存条件 |
Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. |
Important Note |
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
英文介绍 |
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] |