1:Neurobiol. Dis.2012 Aug,47(2)

Prolonged survival and milder impairment of motor function in the SOD1 ALS mouse model devoid of fibroblast growth factor 2.

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by selective motoneuron loss in brain and spinal cord. Mutations in the superoxide dismutase (SOD) 1 gene account...

2:J. Neurol. Sci.2012 Jul 15,318(1-2)

Degenerative myelopathy associated with a missense mutation in the superoxide dismutase 1 (SOD1) gene progresses to peripheral neuropathy in Pembroke Welsh Corgis and Boxers.

Canine degenerative myelopathy (DM) is an adult-onset, fatal neurodegenerative disease with many similarities to an upper-motor-neuron-onset form of human amyotrophic lateral sclerosis (ALS), that res...

3:Mol. Genet. Metab.2012 Jul,106(3)

Allelic variations in superoxide dismutase-1 (SOD1) gene and renal and cardiovascular morbidity and mortality in type 2 diabetic subjects.

Oxidative stress is involved in the pathophysiology of renal and cardiovascular complications of diabetes. Superoxide dismutase (SOD) enzymes play a major role in detoxification of reactive oxygen spe...

4:2012 Jun 14,

Protective Effect of Luteolin in Experimental Ischemic Stroke: Upregulated SOD1, CAT, Bcl-2 and Claudin-5, Down-Regulated MDA and Bax Expression.

Luteolin recently has been proved to elicit a vanity of biological effects through its antioxidant and anti-apoptosis properties. Oxidative and apoptosis damage play an important role in cerebral isch...

5:2012 Jun 13,

Molecular Signatures of Amyotrophic Lateral Sclerosis Disease Progression in Hind and Forelimb Muscles of an SOD1(G93A) Mouse Model.

Abstract Aims: This study utilized proteomics, biochemical and enzymatic assays, and bioinformatics tools that characterize protein alterations in hindlimb (gastrocnemius) and forelimb (triceps) muscl...

6:J. Neurol. Sci.2012 Aug 15,319(1-2)

Distinctive clinicopathological features of 2 large families with amyotrophic lateral sclerosis having L106V mutation in SOD1 gene.

A clinicopathological study of 2 families with familial amyotrophic lateral sclerosis was previously reported [1]. The present study continues to investigate these families, with detailed clinical, ge...

7:2012 Jun 20,

Region-specific changes in the immunoreactivity of TRPV4 expression in the central nervous system of SOD1(G93A) transgenic mice as an in vivo model of amyotrophic lateral sclerosis.

Transient receptor potential vanilloid 4 (TRPV4) is a broadly expressed Ca(2+)-permeable cation channel in the vanilloid subfamily of transient receptor potential channels. It is activated by warm tem...

8:J. Neurosci.2012 Jun 27,32(26)

Targeting of Monomer/Misfolded SOD1 as a Therapeutic Strategy for Amyotrophic Lateral Sclerosis.

There is increasing evidence that toxicity of mutant superoxide dismutase-1 (SOD1) in amyotrophic lateral sclerosis (ALS) is linked to its propensity to misfold and to aggregate. Immunotargeting of di...

9:2012 Jun 29,

Motor terminal degeneration unaffected by activity changes in SOD1(G93A) mice; a possible role for glycolysis.

This study examined whether activity is a contributing factor to motor terminal degeneration in mice that overexpress the G93A mutation of the SOD1 enzyme found in humans with inherited motor neuron d...

10:2012 Jul 3,

Ataxin-3 regulates aggresome formation of copper-zinc superoxide dismutase (SOD1) by editing K63-linked polyubiquitin chains.

Polyubiquitination of misfolded proteins, especially K63-linked polyubiquitination, is thought to be associated with the formation of inclusion bodies. However, it is not well explored whether appropr...

11:Hum. Mol. Genet.2012 Aug 1,21(15)

Mutant superoxide dismutase 1 (SOD1), a cause of amyotrophic lateral sclerosis, disrupts the recruitment of SMN, the spinal muscular atrophy protein to nuclear Cajal bodies.

Spinal muscular atrophy (SMA) and amyotrophic lateral sclerosis (ALS) are among the most common motor neuron diseases to afflict the human population. A deficiency of the survival of motor neuron (SMN...

13:2012 Jul 17,

Morphological and functional changes in innervation of a fast forelimb muscle in SOD1-G85R mice.

Muscle endplates become denervated in mice that express mutations of human superoxide dismutase 1 (hSOD1), models of familial amyotrophic lateral sclerosis. This denervation is especially marked in fa...

14:J. Mol. Biol.2012 Aug 24,421(4-5)

Early Steps in Oxidation-Induced SOD1 Misfolding: Implications for Non-Amyloid Protein Aggregation in Familial ALS.

Among the diseases of protein misfolding, amyotrophic lateral sclerosis (ALS) is unusual in that the proteinaceous neuronal inclusions that are the hallmark of the disease have neither the classic fib...

15:2012 Jul 19,

Mutant SOD1(G93A) Triggers Mitochondrial Fragmentation in Spinal Cord Motor Neurons: Neuroprotection by SIRT3 and PGC-1?

Mutations in the Cu/Zn Superoxide Dismutase (SOD1) gene cause an inherited form of ALS with upper and lower motor neuron loss. The mechanism underlying mutant SOD1-mediated motor neuron degeneration r...

16:Neurobiol. Dis.2012 Oct,48(1)

Selective knockdown of mutant SOD1 in Schwann cells ameliorates disease in G85R mutant SOD1 transgenic mice.

Mutants of superoxide dismutase type 1 (mtSOD1) that have full dismutase activity (e.g., G37R) as well as none (e.g., G85R) cause familial amyotrophic lateral sclerosis (FALS), indicating that mtSOD1-...

17:Neuroscience2012 Sep 18,220

Dantrolene is neuroprotective in vitro, but does not affect survival in SOD1(G93A) mice.

Amyotrophic Lateral Sclerosis (ALS) is a devastating progressive neurodegenerative disease. One of the proposed disease mechanisms is excitotoxicity, in which excessive cytosolic calcium causes neuron...

18:Amyotroph Lateral Scler2012 Sep,13(5)

Marked intrafamilial phenotypic variation in a family with SOD1 C111Y mutation.

Abstract Our objectives were to identify the disease-causing mutation in, and report on the clinical features of, a Japanese family that had coexisting phenotypes of amyotrophic lateral sclerosis and ...

19:Amyotroph Lateral Scler2012 Oct,13(6)

Novel G37V mutation of SOD1 gene in autopsied patient with familial amyotrophic lateral sclerosis.

We report a novel missense mutation (G37V) in exon 2 of the superoxide dismutase-1 gene in a 63-years-old Japanese male with purely lower motor neuron disease. His disease duration was 14 months, and ...

20:Amyotroph Lateral Scler2012 Oct,13(6)

Combined fulminant frontotemporal dementia and amyotrophic lateral sclerosis associated with an I113T SOD1 mutation.

Mutations in the gene for superoxide dismutase type 1 cause amyotrophic lateral sclerosis (ALS), but are not thought to be associated with frontotemporal dementia (FTD). A lack of detailed case report...

Protein Name:SOD1
Gene Name:SOD1


Recombinant Human SOD1/Cu-Zn SOD
(CatNo. CE19)

Recombinant Human Superoxide Dismutase [Cu-Zn]/SOD1 is produced by our E. coli expression system. The target protein is expressed with sequence (Ala2-Gln154) of Human SOD1 fused with a 6His tag at the N-terminus.

Superoxide Dismutase [Cu-Zn] (SOD1) is a soluble cytoplasmic and mitochondrial intermembrane space protein that belongs to the Cu-Zn superoxide dismutase family. SOD1 binds copper and zinc ions and is one of three isozymes responsible for destroying free superoxide radicals in the body. SOD1 neutralizes supercharged oxygen molecules, which can damage cells if their levels are not controlled. The enzyme protects the cell against dangerous levels of superoxide. Zinc binding promotes dimerization and stabilizes the native form. Mutations in SOD1 cause a form of familial amyotrophic lateral sclerosis. Defects in SOD1 are the cause of amyotrophic lateral sclerosis type 1 (ALS1) which is a familial form of amyotrophic lateral sclerosis, a neurodegenerative disorder affecting upper and lower motor neurons and resulting in fatal paralysis.