The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
1/50000. Detects a band of approximately 18 kDa (predicted molecular weight: 16 kDa).
ab172730 - Rabbit monoclonal IgG, is suitable for use as an isotype control with this antibody.
Use at an assay dependent concentration.
1/100 - 1/250.
应用说明Is unsuitable for IP.
功能Destroys radicals which are normally produced within the cells and which are toxic to biological systems.
疾病相关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.
序列相似性Belongs to the Cu-Zn superoxide dismutase family.
翻译后修饰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 reponsible for the non-disulfide-linked homodimerization. Such modification might only occur in extreme conditions and additional experimental evidence is required.
细胞定位Cytoplasm. The pathogenic variants ALS1 Arg-86 and Ala-94 gradually aggregates and accumulates in mitochondria.