製品: SOD1 Antibody
カタログ: DF6077
タンパク質の説明: Rabbit polyclonal antibody to SOD1
アプリケーション: WB IHC
反応性: Human, Mouse, Rat
予測: Pig, Bovine, Horse, Sheep, Rabbit, Dog
分子量: 20kD; 16kD(Calculated).
ユニプロット: P00441
RRID: AB_2838045

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製品説明

ソース:
Rabbit
アプリケーション:
WB 1:500-1:2000, IHC 1:50-1:200
*The optimal dilutions should be determined by the end user.
*Tips:

WB: For western blot detection of denatured protein samples. IHC: For immunohistochemical detection of paraffin sections (IHC-p) or frozen sections (IHC-f) of tissue samples. IF/ICC: For immunofluorescence detection of cell samples. ELISA(peptide): For ELISA detection of antigenic peptide.

反応性:
Human,Mouse,Rat
予測:
Pig(88%), Bovine(100%), Horse(100%), Sheep(100%), Rabbit(100%), Dog(88%)
クローナリティ:
Polyclonal
特異性:
SOD1 Antibody detects endogenous levels of total SOD1.
RRID:
AB_2838045
引用形式: Affinity Biosciences Cat# DF6077, RRID:AB_2838045.
コンジュゲート:
Unconjugated.
精製:
The antiserum was purified by peptide affinity chromatography using SulfoLink™ Coupling Resin (Thermo Fisher Scientific).
保存:
Rabbit IgG in phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol. Store at -20 °C. Stable for 12 months from date of receipt.
別名:

折りたたみ/展開

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;

免疫原

免疫原:
Uniprot:
遺伝子(ID):
タンパク質の説明:
SOD1, Cu/Zn superoxide dismutase, is a major antioxidant enzyme that catalyzes the conversion of superoxide anion to hydrogen peroxide and molecular oxygen (1). SOD1 is ubiquitously expressed and is localized in the cytosol, nucleus and mitochondrial intermembrane space. The SOD1 gene locus is on chromosome 21 in a region affected in Down Syndrome (2). In addition, over 100 distinct SOD1 inherited mutations have been identified in the familial form of amyotrophic lateral sclerosis (ALS), a progressive degenerative disease of motor neurons (3-5). Despite the fact that SOD1 helps to eliminate toxic reactive species, its mutations in ALS have been described as gain-of-function (5). The mechanism by which mutant SOD1 induces the neurodegeneration observed in ALS is still unclear. Mutant SOD1 proteins become misfolded and consequently oligomerize into high molecular weight species that aggregate and end up in proteinaceous inclusions (5).
タンパク質配列:
MATKAVCVLKGDGPVQGIINFEQKESNGPVKVWGSIKGLTEGLHGFHVHEFGDNTAGCTSAGPHFNPLSRKHGGPKDEERHVGDLGNVTADKDGVADVSIEDSVISLSGDHCIIGRTLVVHEKADDLGKGGNEESTKTGNAGSRLACGVIGIAQ

種類予測

種類予測:

Score>80(red) has high confidence and is suggested to be used for WB detection. *The prediction model is mainly based on the alignment of immunogen sequences, the results are for reference only, not as the basis of quality assurance.

Species
Results
Score
Horse
100
Bovine
100
Sheep
100
Rabbit
100
Pig
88
Dog
88
Xenopus
75
Zebrafish
75
Chicken
75
Model Confidence:
High(score>80) Medium(80>score>50) Low(score<50) No confidence

PTMs - P00441 基板として

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

機能:

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

PTMs:

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.

細胞の位置付け:

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
サブユニット構造:

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.

タンパク質ファミリー:

Belongs to the Cu-Zn superoxide dismutase family.

研究領域

· 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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