Product: SOD1 Mouse Monoclonal Antibody
Catalog: BF8509
Description: Mouse monoclonal antibody to SOD1
Application: WB IF/ICC
Reactivity: Human, Mouse, Rat
Prediction: Pig, Bovine, Horse, Sheep, Rabbit, Dog, Chicken
Mol.Wt.: 16 Kd; 16kD(Calculated).
Uniprot: P00441

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 100ul $280 In stock
 200ul $350 In stock

Lead Time: Same day delivery

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Product Info

Source:
Mouse
Application:
WB 1:500-1:3000, IF/ICC 1:100-1:500
*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.

Reactivity:
Human,Mouse,Rat
Clonality:
Monoclonal [AFfirm8509]
Conjugate:
Unconjugated.
Purification:
Affinity-chromatography.
Storage:
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.
Alias:

Fold/Unfold

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;

Immunogens

Immunogen:
Uniprot:
Gene(ID):
Description:
Destroys radicals which are normally produced within the cells and which are toxic to biological systems.
Sequence:
MATKAVCVLKGDGPVQGIINFEQKESNGPVKVWGSIKGLTEGLHGFHVHEFGDNTAGCTSAGPHFNPLSRKHGGPKDEERHVGDLGNVTADKDGVADVSIEDSVISLSGDHCIIGRTLVVHEKADDLGKGGNEESTKTGNAGSRLACGVIGIAQ

PTMs - P00441 As Substrate

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

Research Backgrounds

Function:

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.

Subcellular Location:

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
Subunit Structure:

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.

Family&Domains:

Belongs to the Cu-Zn superoxide dismutase family.

Research Fields

· 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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For Research Use Only.
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