产品编号 30-10216r-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.
说 明 书 100ul  
研究领域
抗体来源 rabbit
克隆类型 polyclonal
交叉反应 human, mouse, rat, pig, cow, horse, 
产品应用 icc=1:50-200 if=1:50-200  
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
亚    型 igg
纯化方法 affinity purified by protein a
储 存 液 preservative: 15mm sodium azide, constituents: 1% bsa, 0.01m pbs, ph 7.4
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.

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