Clumps of Neuron Killing Proteins Implicated in Neurodegenerative Disorders

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Highlights

• Scientists have identified that mutation in SOD1 leads to clumping of protein that destroys neurons in the brain.
• The destruction of neurons leads to paralysis and death in Amyotrophic Lateral Sclerosis (ALS) and other neurodegenerative diseases.
• Phosphorylation of SOD1 prevents clumping and death of neurons.
• Study provides new targets for drug therapy.

Scientists from the UNC school of Medicine have shown that clumps of protein present in the brain and the spinal cord could lead to the death of neurons by blocking their functioning. This discovery is highly significant in neurodegenerative diseases like Amyotrophic Lateral Sclerosis (ALS) which has no known cure.

The UNC researchers have found that the malfunctioning proteins can be reversed in a first every study of this kind. The scientists stabilized SOD1 by demonstrating that mutation of disease causing SOD1 could stabilize it.

‘Phosphorylation of SOD1 can prevent death of neurons in ALS.’

SOD1 and ALS

Amyotrophic Lateral Sclerosis (ALS) is a neurodegenerative disorder that is fatal and studies have identified that mutation in the gene that encodes Copper-Zinc superoxide dismutase (SOD1) could lead to a large percentage of the disease condition.

The mutated SOD1 is found to differ from the wild type in the following ways:

• Structure
• Stability
• Activity
• Higher propensity to aggregate

Oxidative Damage Caused Due to Mutant SOD1

The native form of SOD1 was found to react with superoxide and result in hydrogen peroxide and oxygen radicals, lowering the effect of the superoxide. However, mutations in SOD1 could result in the reversal of the process, with hydrogen peroxide converted to superoxide.

Aggregation of SOD1

Mutant SOD1 in ALS has a greater propensity to form clumps that can lead to the damage of neurons in the brain. The ability of mutated SOD1 to form clumps was determined but the understanding that these clumps could lead to disease propagation was understood from the study conducted by Ayers JI et all, the study published in the Journal Acta Neuropathologica (2014). The researchers injected spinal cord homogenates into mice and found that the aggregates spread and lead to the disease.

(http://www.ncbi.nlm.nih.gov/pubmed/25262000)Source: Medindia

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