Provided are novel human copper-zinc superoxide dismutase, also known as superoxide dismutase 1 or SOD1, specific antibodies as well as fragments, derivatives and variants thereof as well as methods related thereto. Assays, kits, and solid supports related to antibodies specific for SOD1 are also disclosed. The antibody, immunoglobulin chain(s), as well as binding fragments, derivatives and variants thereof can be used in pharmaceutical and diagnostic compositions for SOD1 targeted immunotherapy and diagnosis, respectively.

The invention relates to inhibitory nucleic acids and rAAV-based compositions, methods and kits useful for treating Amyotrophic Lateral Sclerosis.

Formulation(s) of prooxidation agents and/or antioxidant capacity reducing agents for producing electronically modified oxygen derivatives (EMODs) for cancer chemo-suppression and chemo-protection, and kit(s) and method(s) of use thereof.

The invention discloses a novel high-temperature resistant superoxide dismutase (SOD) and a coding gene and application thereof. The superoxide dismutase has a very high stability and activity under various severe conditions such as high temperature, high acidity or alkalinity and various proteases such as pepsin and trypsin. The superoxide dismutase of the present invention overcomes the problems of the existing SOD products, such as unstability, low in activity or even a complete lack of activity and prone to degradation and inactivation in the digestive tract. And the superoxide dismutase of the present invention is expected to be developed into cosmetics or health-care foods or medicines which are stable in activity and have a good effect.

Disclosed herein are antisense compounds and methods for decreasing SOD-1 mRNA and protein expression. Such methods, compounds, and compositions are useful to treat, prevent, or ameliorate SOD-1 associated diseases, disorders, and conditions. Such SOD-1 associated diseases include amyotrophic sclerosis (ALS).

The present invention provides methods of enhancing the efficacy and specificity of RNAi using single or double blunt-ended siRNA. The invention also provides single and double-blunt ended siRNA compositions, vectors, and transgenes containing the same for mediating silencing of a target gene. Therapeutic methods are also featured.

Described herein are compounds and methods for tethering proteins. For example, dimers of proteins, including SOD1 and DJ-1, are described, where the dimers are formed by the covalent bonding of a cysteine on the first monomer to a cysteine on the second monomer via a cyclic disulfide linker. The covalently attached dimers exhibit increased stabilization.

The disclosure relates to double stranded ribonucleic acid (dsRNAi) agents and compositions targeting a SOD1 gene, as well as methods of inhibiting expression of a SOD1 gene and methods of treating subjects having a SOD1-associated neurodegenerative disease or disorder, e.g., Amyotrophic Lateral Sclerosis (ALS), Alzheimer's disease (AD), Parkinson's disease (PD), and Down's syndrome (DS), using such dsRNAi agents and compositions.

Provided herein are isotopically labeled reagents, including isotopically labeled small molecules and peptides, that can be used to detect and/or quantify -N-methylamino-L-alanine (BMAA) in the Cu/Zn Superoxide Dismutase 1 (SOD1) protein. Further provided are methods for detecting, preventing, or treating amyotrophic lateral sclerosis in a subject using isotopically labeled reagents to detect and/or quantify -N-methylamino-L-alanine (BMAA) in the Cu/Zn Superoxide Dismutase 1 (50D1) protein. Further provided are isotopically labeled reagents and methods for detecting BMAA in additional proteins from patient samples.

The present invention relates to a use of earthworm protein. Particularly, the present invention provides a use of earthworm protein for preparing a pharmaceutical composition which can enhance the expression of an antioxidant gene in brain neurons or inhibit the effects of -amyloid protein (A) and 1-methyl-4-phenylpyridinium (MPP.sup.+) on brain neurons. As such, the pharmaceutical composition comprising the earthworm protein has effects of reducing damage to brain neurons.