Table 1.
Neuroprotective actions of cysteamine/cystamine.
| Cytoprotective effects of Cysteamine/Cystamine | System | References |
|---|---|---|
| Protection against glutamate-induced toxicity | Primary glial cells | (25) |
| Scavenges acrolein, a toxic metabolite generated during lipid peroxidation. Drug detoxification and polyamine oxidation | Cysteamine: Acetaminophen-induced hepatic injury in mice | (26) |
| Reduces oxidative stress and antioxidant balance in regulatory T cells | Cystamine: systemic lupus erythematosus (SLE)-prone mice | (27) |
| Improved membrane functionality, reduced lipid peroxidation and improved viability of sperm | Cysteamine: Cyropreserved Ram semen | (28) |
| Intraperitoneal injection of cystamine mediates neuroprotection by enhancing neuronal progenitor cell proliferation and proliferation through the BDNF pathway | Cystamine: mouse model of stroke | (29) |
| Dopaminergic neurodegeneration induced by MPTP is prevented by cysteamine and cystamine | MPTP model of neurodegeneration | (30, 31) |
| Neuroprotection from 3-nitropropionic acid (3NP) toxicity by cystamine | Stimulates NF-E2 related factor 2 (Nrf2) signaling in cell culture and the 3-NP model of neurodegeneration in mice | (32) |
| Administration of cystamine confers protection against haloperidol-induced toxicity and ischemic brain injury | Mouse model | (33) |
| Aggregation of amyloid β1−42 (Aβ) in astrocyte cultures reduced by cystamine | Cultured astrocystes | (34) |
| Cystamine elevated L-cysteine levels in HD | R6/2 mouse model of HD and PC12 model of polyglutamine aggregation | (35) |
| Transglutaminase-induced aggregation of alpha-synuclein decreased by cystamine | in vitro and in COS-7 cells | (36) |
| Cystamine significantly extended survival, improved body weight and motor performance, and delayed the neuropathological sequela | R6/2 mouse model of HD | (37) |
| Cystamine increased viability of striatal progenitor cells harboring mutant huntingtin and prevented ROS formation in HD cells subjected to H2O2 and STS | STHdhQ7/Q7 and STHdhQ111/Q111 striatal progenitor cell lines | (38) |
| Cysteamine and cystamine prevented the 3-NP-mediated decrease in cellular and mitochondrial GSH levels as well as mitochondrial depolarization | STHdhQ7/Q7 and STHdhQ111/Q111 striatal progenitor cell lines | (39) |
| Cystamine extended survival, reduced associated tremor and abnormal movements and ameliorated weight loss. Increased the transcription of the chaperone HDJ1/Hsp40 | R6/2 mouse model of HD | (40) |
| Cystamine significantly delayed the progression of ALS symptoms and reduced SOD1 oligomers and microglial activation | G93A mouse model of ALS, cell culture models | (41) |
| Cystamine prevents toxicity induced by aggregation of polyadenylate-binding protein nuclear 1 | Mouse model of Oculopharyngeal muscular dystrophy (OPMD) | (42) |
| Cystamine modulates blood pressure and reduces hypertension | Spontaneously hypertensive rats | (43) |
| Cysteamine alleviates fibrosis and symptoms associated with chronic kidney disease (CKD) | Mouse models of CKD | (44) |
| Cysteamine suppresses cataract formation induced by selenite | Rats | (45) |
| Cystamine rescued behavioral deficits induced by 2,5-hexanedione by increasing BDNF and hsp70 expression | Rats | (46) |