产品: SOD1 小鼠 单克隆 抗体
货号: BF8509
描述: Mouse monoclonal antibody to SOD1
应用: WB IF/ICC
反应: Human, Mouse, Rat
预测: Pig, Bovine, Horse, Sheep, Rabbit, Dog, Chicken
分子量: 16 Kd; 16kD(Calculated).
蛋白号: P00441

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   规格 价格 库存
 50ul RMB¥ 1250 现货
 100ul RMB¥ 2300 现货
 200ul RMB¥ 3000 现货

货期: 当天发货

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产品描述

来源:
Mouse
应用:
WB 1:500-1:3000, IF/ICC 1:100-1:500
*The optimal dilutions should be determined by the end user.
*Tips:

WB: 适用于变性蛋白样本的免疫印迹检测. IHC: 适用于组织样本的石蜡(IHC-p)或冰冻(IHC-f)切片样本的免疫组化/荧光检测. IF/ICC: 适用于细胞样本的荧光检测. ELISA(peptide): 适用于抗原肽的ELISA检测.

反应:
Human,Mouse,Rat
克隆:
Monoclonal [AFfirm8509]
偶联:
Unconjugated.
纯化:
Affinity-chromatography.
保存:
Mouse IgG1 in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol. Store at -20 °C. Stable for 12 months from date of receipt.
别名:

展开/折叠

ALS; ALS1; Amyotrophic lateral sclerosis 1 adult; Cu/Zn SOD; Cu/Zn superoxide dismutase; Epididymis secretory protein Li 44; HEL S 44; Homodimer; hSod1; Indophenoloxidase A; IPOA; Mn superoxide dismutase; SOD; SOD soluble; SOD1; SOD2; SODC; SODC_HUMAN; Superoxide dismutase [Cu-Zn]; Superoxide dismutase 1; Superoxide dismutase 1 soluble; Superoxide dismutase Cu Zn; Superoxide dismutase cystolic;

抗原和靶标

免疫原:
Uniprot:
基因/基因ID:
描述:
Destroys radicals which are normally produced within the cells and which are toxic to biological systems.
序列:
MATKAVCVLKGDGPVQGIINFEQKESNGPVKVWGSIKGLTEGLHGFHVHEFGDNTAGCTSAGPHFNPLSRKHGGPKDEERHVGDLGNVTADKDGVADVSIEDSVISLSGDHCIIGRTLVVHEKADDLGKGGNEESTKTGNAGSRLACGVIGIAQ

翻译修饰 - P00441 作为底物

Site PTM Type Enzyme
A2 Acetylation
T3 Phosphorylation
K4 Ubiquitination
C7 S-Nitrosylation
K10 Acetylation
K10 Ubiquitination
K24 Acetylation
K24 Ubiquitination
S26 Phosphorylation
K31 Ubiquitination
S35 Phosphorylation
K37 Ubiquitination
T40 Phosphorylation
T59 Phosphorylation
S60 Phosphorylation
S69 Phosphorylation
K71 Acetylation
K71 Ubiquitination
K76 Sumoylation
K76 Ubiquitination
R80 Methylation
T89 Phosphorylation
K92 Ubiquitination
S99 Phosphorylation
S103 Phosphorylation
S106 Phosphorylation
S108 Phosphorylation
K123 Acetylation
K123 Ubiquitination
K129 Ubiquitination
K137 Ubiquitination
R144 Methylation
C147 S-Nitrosylation

研究背景

功能:

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

翻译修饰:

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.

Palmitoylation helps nuclear targeting and decreases catalytic activity.

Succinylation, adjacent to copper catalytic site, probably inhibits activity. Desuccinylation by SIRT5 enhances activity.

细胞定位:

Cytoplasm. Mitochondrion. Nucleus.
Note: Predominantly cytoplasmic; the pathogenic variants ALS1 Arg-86 and Ala-94 gradually aggregates and accumulates in mitochondria.

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionSubcellular location
亚基结构:

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.

蛋白家族:

Belongs to the Cu-Zn superoxide dismutase family.

研究领域

· Cellular Processes > Transport and catabolism > Peroxisome.   (View pathway)

· Human Diseases > Neurodegenerative diseases > Amyotrophic lateral sclerosis (ALS).

· Human Diseases > Neurodegenerative diseases > Huntington's disease.

· Human Diseases > Neurodegenerative diseases > Prion diseases.

· Organismal Systems > Aging > Longevity regulating pathway - multiple species.   (View pathway)

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