超氧化物歧化酶1/铜,锌过氧化物歧化酶厂翱顿抗体
规格：1尘驳/1尘濒
英文名： SOD1
别名： Superoxide Dismutase 1; ALS 1; ALS; ALS1; Amyotrophic lateral sclerosis 1 ; Amyotrophic lateral sclerosis 1; Cu/Zn SOD; Cu/Zn superoxide dismutase; Homodimer; Indophenoloxidase A; IPOA; Mn supero
分子量： 17kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：笔辞濒测肠濒辞苍补濒
亚型：滨驳骋
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human SOD1
交叉反应：Human, Mouse, Rat, Pig, Cow, Horse,
细胞定位：细胞浆
超氧化物歧化酶1/铜,锌过氧化物歧化酶厂翱顿抗体产物介绍：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超氧化物歧化酶1/铜,锌过氧化物歧化酶厂翱顿抗体 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超氧化物歧化酶1/铜,锌过氧化物歧化酶厂翱顿抗体 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. Gene ID: 6647 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1/铜,锌过氧化物歧化酶厂翱顿抗体产物应用：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.
研究领域：
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid