Oxidation-induced Misfolding and Aggregation of Superoxide Dismutase and Its Implications for Amyotrophic Lateral Sclerosis*

The presence of intracellular aggregates that contain Cu/Zn superoxide dismutase (SOD1) in spinal cord motor neurons is a pathological hallmark of amyotrophic lateral sclerosis (ALS). Although SOD1 is abundant in all cells, its half-life in motor neurons far exceeds that in any other cell type. On the basis of the premise that the long half-life of the protein increases the potential for oxidative damage, we investigated the effects of oxidation on misfolding/aggregation of SOD1 and ALS-associated SOD1 mutants. Zinc-deficient wild-type SOD1 and SOD1 mutants were extremely prone to form visible aggregates upon oxidation as compared with wild-type holo-protein. Oxidation of select histidine residues that bind metals in the active site mediates SOD1 aggregation. Our results provide a plausible model to explain the accumulation of SOD1 aggregates in motor neurons affected in ALS.

• Publication year

  2002

• Venue

  Journal of Biological Chemistry

• Publication date

  2002-12-06

• Fields of study

  Biology, Medicine, Chemistry

• Identifiers
  DOI 10.1074/JBC.M207356200PMID 12356748
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• These findings support a plausible model for the accumulation of SOD1 aggregates in spinal cord motor neurons affected in amyotrophic lateral sclerosis (ALS).

  Confidence 0.92

  박진우 (dztg5apj7m) extraction뀨 (7c402c1b98) reviewB (s683577b42) reviewq (76h6bfydm6) reviewAK (4715169a40) reviewKiller Whale (322360f1c1) reviewmexicorea (qjvnbu8xg3) review
• Oxidation of select histidine residues that coordinate metals in the active site mediates SOD1 aggregation.

  Confidence 0.97

  박진우 (dztg5apj7m) extraction뀨 (7c402c1b98) reviewB (s683577b42) reviewq (76h6bfydm6) reviewAK (4715169a40) reviewKiller Whale (322360f1c1) reviewmexicorea (qjvnbu8xg3) review
• Zinc-deficient wild-type SOD1 and ALS-associated SOD1 mutants were highly prone to visible aggregate formation upon oxidation compared with wild-type holo-protein.

  Confidence 0.98

  박진우 (dztg5apj7m) extraction뀨 (7c402c1b98) reviewB (s683577b42) reviewq (76h6bfydm6) reviewAK (4715169a40) reviewKiller Whale (322360f1c1) reviewmexicorea (qjvnbu8xg3) review

• active site

  structural regions

  The metal-binding catalytic region of SOD1 discussed as the oxidation-sensitive structural site.

  Aliases: metal-binding site

  박진우 (dztg5apj7m) extraction뀨 (7c402c1b98) reviewB (s683577b42) reviewq (76h6bfydm6) reviewAK (4715169a40) reviewKiller Whale (322360f1c1) reviewmexicorea (qjvnbu8xg3) review
• amyotrophic lateral sclerosis (als)

  diseases

  A neurodegenerative disease associated here with SOD1 aggregate accumulation in motor neurons.

  Aliases: ALS

  박진우 (dztg5apj7m) extraction뀨 (7c402c1b98) reviewB (s683577b42) reviewq (76h6bfydm6) reviewAK (4715169a40) reviewKiller Whale (322360f1c1) reviewmexicorea (qjvnbu8xg3) review
• cu/zn superoxide dismutase (sod1)

  protein

  A copper-zinc superoxide dismutase enzyme that serves as the protein examined for oxidation-driven misfolding and aggregation.

  Aliases: SOD1

  박진우 (dztg5apj7m) extraction뀨 (7c402c1b98) reviewB (s683577b42) reviewq (76h6bfydm6) reviewAK (4715169a40) reviewKiller Whale (322360f1c1) reviewmexicorea (qjvnbu8xg3) review
• histidine residues

  amino acid residues

  Histidine amino acid residues in SOD1 that participate in metal coordination at the active site.

  Aliases: His residues

  박진우 (dztg5apj7m) extraction뀨 (7c402c1b98) reviewB (s683577b42) reviewq (76h6bfydm6) reviewAK (4715169a40) reviewKiller Whale (322360f1c1) reviewmexicorea (qjvnbu8xg3) review
• intracellular aggregates

  aggregate structures

  Protein aggregates that form inside cells and are observed here as visible SOD1-containing clumps.

  Aliases: aggregates

  박진우 (dztg5apj7m) extraction뀨 (7c402c1b98) reviewB (s683577b42) reviewq (76h6bfydm6) reviewAK (4715169a40) reviewKiller Whale (322360f1c1) reviewmexicorea (qjvnbu8xg3) review
• oxidation

  process

  A chemical modification process involving electron loss that was applied to SOD1 in the experiments.

  박진우 (dztg5apj7m) extraction뀨 (7c402c1b98) reviewB (s683577b42) reviewq (76h6bfydm6) reviewAK (4715169a40) reviewKiller Whale (322360f1c1) reviewmexicorea (qjvnbu8xg3) review
• sod1 mutants

  protein variants

  ALS-associated variant forms of SOD1 analyzed alongside wild-type protein.

  Aliases: ALS-associated SOD1 mutants

  박진우 (dztg5apj7m) extraction뀨 (7c402c1b98) reviewB (s683577b42) reviewq (76h6bfydm6) reviewAK (4715169a40) reviewKiller Whale (322360f1c1) reviewmexicorea (qjvnbu8xg3) review
• spinal cord motor neurons

  cell types

  The neuron type in which SOD1 aggregates are described as a pathological hallmark of disease.

  Aliases: motor neurons

  박진우 (dztg5apj7m) extraction뀨 (7c402c1b98) reviewB (s683577b42) reviewq (76h6bfydm6) reviewAK (4715169a40) reviewKiller Whale (322360f1c1) reviewmexicorea (qjvnbu8xg3) review
• wild-type holo-protein

  protein form

  The metal-loaded wild-type SOD1 form used as the comparison state in the experiments.

  Aliases: wild-type holo SOD1

  박진우 (dztg5apj7m) extraction뀨 (7c402c1b98) reviewB (s683577b42) reviewq (76h6bfydm6) reviewAK (4715169a40) reviewKiller Whale (322360f1c1) reviewmexicorea (qjvnbu8xg3) review
• zinc-deficient wild-type sod1

  protein form

  The wild-type SOD1 protein form lacking zinc bound at its metal-binding site.

  Aliases: zinc-deficient SOD1

  박진우 (dztg5apj7m) extraction뀨 (7c402c1b98) reviewB (s683577b42) reviewq (76h6bfydm6) reviewAK (4715169a40) reviewKiller Whale (322360f1c1) reviewmexicorea (qjvnbu8xg3) review

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