Aggregation modulating elements in mutant human superoxide dismutase 1

Human Erythrocyte Superoxide Dismutase Encapsulated in Positively Charged Liposomes

      Superoxide dismutase (SOD) is an important antioxidant that protects many types of cells from the free radical damage. One of the possible ways for the use of SOD is its incorporation in liposomes. The aim of this study was to investigate the effect of cationic phospholipids on the entrapment of human erythrocyte superoxide dismutase (Cu/Zn SOD) in liposomes. Also, in the present study, w...

Mutant superoxide dismutase-1 indistinguishable from wild-type causes ALS.

A reason for screening amyotrophic lateral sclerosis (ALS) patients for mutations in the superoxide dismutase-1 (SOD1) gene is the opportunity to find novel mutations with properties that can give information on pathogenesis. A novel c.352C>G (L117V) SOD1 mutation was found in two Syrian ALS families living in Europe. The disease showed unusually low penetrance and slow progression. In erythroc...

Superoxide dismutase Superoxide dismutase

Cupric Ions Induce the Oxidation and Trigger the Aggregation of Human Superoxide Dismutase 1

BACKGROUND Amyotrophic lateral sclerosis (ALS), partly caused by the mutations and aggregation of human copper, zinc superoxide dismutase (SOD1), is a fatal degenerative disease of motor neurons. Because SOD1 is a major copper-binding protein present at relatively high concentration in motor neurons and copper can be a harmful pro-oxidant, we want to know whether aberrant copper biochemistry co...

An intersubunit disulfide bond prevents in vitro aggregation of a superoxide dismutase-1 mutant linked to familial amytrophic lateral sclerosis.

Familial amyotrophic lateral sclerosis (FALS) is linked to over 90 point mutations in superoxide dismutase-1 (SOD1), a dimeric metalloenzyme. The postmortem FALS brain is characterized by SOD1 inclusions in the motor neurons of regions in which neuronal loss is most significant. These findings, together with animal modeling studies, suggest that aggregation of mutant SOD1 produces a pathogenic ...