Antibodies for Amyotrophic Lateral Sclerosis - FocusOn 133
Amyotrophic Lateral Sclerosis (ALS) is described as a neurodegenerative disease and characterized by degeneration of upper and lower motor neurons. ALS is specified by mid-to-late-life onset, selective neuronal death and the formation of protein deposits in affected neuronal tissues similar to other neurodegenerative diseases (Alzheimer's disease: FocusOn 130, Parkinson's disease: FocusOn 131, Huntington's disease: FocusOn 132). Sporadic cases of ALS occur with no family history of ALS. Familial ALS is due to inherited mutations spread throughout the SOD1 polypeptide.
Superoxide Dismutase (SOD)
SOD is an antioxidant enzyme and as such part of the defense system against reactive oxygen species. SOD catalyses the dismutation reaction of the superoxide radical anion (O2-) to hydrogen peroxide (H2O2) through glutathione reductase and catalase. Several classes of SODs have been identified, which include the intracellular copper/zinc SOD (Cu/Zn-SOD, SOD1), the mitochondrial manganese SOD (Mn-SOD, SOD2) and the extracellular copper/zinc SOD (ECSOD, SOD3).
SOD1 is found in all eukaryotic species as a homodimeric 32 kDa enzyme, which contains one Cu- and one Zn-ion each. SOD1 neutralizes supercharged oxygen molecules (superoxide radicals), which can damage cells if their levels are not controlled. The mechanism through which mutant SOD1 causes ALS is not known, but instability, misfolding and aggregation of mutant SOD1 is hypothesized. Biochemical markers of SOD1 misfolding have been noticed as early indicators of ALS. One such marker is the appearance of detergent insoluble precipitates of SOD1 which correlate well with disease onset and progression.

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TAR DNA-binding Protein 43 (TARDBP, TDP-43)
The cellular protein TARDBP functions as a DNA-binding protein and specifically binds to the TAR DNA sequence motifs of HIV. TARDBP protein has been identified as a major pathological protein of sporadic and familial frontotemporal lobar degeneration with ubiquitin-positive, tau-negative inclusions with or without motor neuron disease. Therefore, TARDBP defines a novel class of neurodegenerative diseases called TARDBP proteinopathies.

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Key References
- Cairns NJ et al, Jul 2007, Am J Pathol 171(1): 227-240
- Eisen A et al, Apr 2008, Amyotroph Lateral Scler 9(2): 108-119
- Geser F et al, Feb 2009, Arch Neurol 66(2): 180-189
- Jonsson PA et al, Jan 2004, Brain 127(Pt 1): 73-88
- Koyama S, 2006, Biochem Biophys Res Commun 343(3): 719-30
- Pesirids, GS et al, Oct 2009, Hum Mol Genet 18(R2): R156-162
- Proescher, JB et al, Jun 2008, Hum Mol Genet 17(12): 1728-1737
- Valentine, JS et al, Apr 2003, Proc Natl Acad Sci USA100(7): 3617-3622
Please refer also to other FocusOns in this series about Neurosciences
- FocusOn 130: Antibodies to Alzheimer’s Disease
- FocusOn 131: Antibodies to Parkingson’s Disease
- FocusOn 132: Antibodies to Huntington’s Disease
- FocusOn 133: Antibodies to Amyotrophic Lateral Sclerosis
Primary Antibodies
Catalog No. | Host | Iso. | Clone | Pres. | React. | Applications | |
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AM01230PU-N | Superoxide Dismutase 1 / SOD1 antibody |
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Mouse | IgG1 | 2F5 | Purified | Bov, Hu, Ms | C, E, P |
0.1 mg /
$330.00
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OriGene Technologies GmbH | |||||||
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AP00192PU-N | Superoxide Dismutase 1 / SOD1 antibody |
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Rabbit | Aff - Purified | Eq, GP, Hu, Ms, Rt | C, IP, P, WB |
0.1 mg /
$365.00
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OriGene Technologies GmbH | |||||||
AP18205PU-N | Superoxide Dismutase 1 / SOD1 (Center) antibody |
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Rabbit | Hu | WB |
0.4 ml /
$415.00
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OriGene Technologies GmbH | |||||||
R1111PS | Superoxide Dismutase 1 / SOD1 antibody |
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Rabbit | IgG | Aff - Purified | Bov | E, ICC/IF, ID, IP, R, WB |
1 mg /
$530.00
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OriGene Technologies GmbH | |||||||
AM09038PU-S | TARDBP antibody |
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Mouse | IgG1 | k1B8 | Purified | Hu | E, P, WB |
50 µl /
$220.00
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OriGene Technologies GmbH | |||||||
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AP08534PU-N | TARDBP antibody |
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Rabbit | IgG | Purified | Hu, Ms, Rt | P, WB |
50 µg /
$425.00
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OriGene Technologies GmbH |