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. 2020 Apr 20;48(2):379–397. doi: 10.1042/BST20190054

Table 1. Summary of redox-regulated protein aggregates.

Protein Abbreviation Normal function Involved residue (Cys) Modification Consequence(s) Type of aggregation Related pathologies Reference
AMP-activated protein kinase AMPK-alpha Energy metabolism C130 and C174 Disulfide Inactivation Soluble aggregates Cardiopathologies, energy starvation [95]
Apolipoprotein A-I APOA1 Cholesterol transport Methionines Methionine oxidation Partial unfolding, fibrillization and inactivation Amyloid APOA1 amyloidoses and atherosclerosis [94]
APP/Amyloid-β Unknown M35 Methionine oxidation Required for pro-oxidative activity Amyloid Alzheimer's [119]
Ataxin-2 ATXN2 TORC1 inhibition Methionines Methionine oxidation Oxidation reverses aggregation, functional regulation of activity LLPS, amyloid Spinocerebellar atrophy, ALS [80]
Cellular prion protein PrPC and PrPSC Synaptic function C179 and C214 Disulfide exchange Reduction of PrPC induces aggregation of PrPSC polymer Amyloid Transmissible spongiform encephalopathies [96]
Cyclin-dependent kinase inhibitor 2A p16INK4A Cell cycle regulation, senescence C72 Homodimerisation Inactivation Amyloid Cancer [69]
Endostatin COL18A1 Angiogenesis inhibition C33, C135, C165 and C173 Disulfide Prevents aggregation Amyloid Alzheimer's [105]
Fas ligand FasL Apoptosis, inflammation Methionines Methionine oxidation Multimerization and aggregation enhanced biological activity Unknown Acute lung injury [92]
Glyceraldehyde-3-phosphate dehydrogenase GAPDH Glycolysis M46 Methionine oxidation, disulfides Local conformational change promotes disulfide cross-linking and aggregation Amyloid Alzheimer's, motor neuron disease [87]
Growth hormone (recombinant) hGH (Therapeutic protein production) M14 and M125 Methionine oxidation Lower stability Unknown GH deficiency [90]
human Islet Amyloid Polypeptide hIAPP Insulin/glucagon secretion, gastric emptying C2 and C7 Disulfide Prevents aggregation Amyloid Type 2 diabetes [103]
Huntingtin HTT Unknown M8 Post-aggregation methionine oxidation Controls interaction between aggregates Amyloid Huntington disease [106]
C115 and C119 Disulfide mediated oligomerization Oxidation-dependent soluble toxic oligomers, slower clearance Soluble oligomeric aggregates [97]
Interferon-β1a (recombinant) IFNβ1a (Therapeutic protein production) Many residues (M, H, F, W, Y) Cross-linking Lower stability Unknown [89]
Mitochondrial GrpE protein homologue MGE1 Proteostasis, HSP70 cochaperone M155 methionine oxidation Inactive HSP70 but targeting it to unfolded proteins Amyloid Myopathies [141]
Sequestosome-1 SQSTM1/p62 Autophagy C105 and C113 Disulfide mediated oligomerization More autophagy, cell survival Insoluble aggregates, LLPS ALS [170]
Superoxide dismutase 1 SOD1 Dismutation of superoxide C111 Disulfide-linked dimerization Oligomerization and subsequent fibril formation Oligomeric, amyloid and amorphous ALS [102]
Transthyretin TTR Thyroid hormone binding C10, M1 and M13 Cysteine sulfonic acid, methionine sulfoxide Tetramer dissociation and aggregation Amyloid Senile systemic amyloidosis [93]
Tryptophan hydroxylase 2 TPH2 Serotonin biosynthesis Any out of 13 cysteines Disulfide, cross-linking Misfolding, intra- and intermolecular disulfide bond formation, protein inactivation Unknown, disulfide cross-linked oligomers Parkinson's [72]
Vinisin-like protein 1 VSNL1 Calcium sensing C187 Disulfide-linked homodimerization Reduced levels of functional protein Amyloid, disulfide cross-linked oligomers ALS, AD [75]
y-synuclein SNCG Neurofilament network integrity M38 and Y39 Oxidation-dependent oligomerization Aggregation and seeding for α-synuclein aggregation Amyloid Parkinson's [88]
β2-microglobulin β2M MHCI light chain C25 and C80 Disulfide reduction, disulfide exchange, post-aggregation oxidation Aggregation, post-aggregation stabilization Amyloid Hemodialysis-related amyloidosis [107]
γ-crystallins CRYG Lens transparency C32 and C41 Intramolecular disulfide Destabilizes its N-terminal domain, stabilizes an intermediate which is prone to aggregation Amorphous Cataract [82]
κ-casein CSN3 Milk protein M95 and M106 Methionine oxidation Increased aggregation, increased toxicity Amyloid Corpora amylacea [91]