Table 1.
List of proteins affected by CL dysfunction.
| Protein Affected | Mitochondrial Alterations | Biological Sample | Ref. |
|---|---|---|---|
| MIC10 subunit | -Cristae defects -Misdistribution of MRC |
-Yeast -Hela, HepG2 cells |
[29,30,31] |
| F1Fo ATP synthase | -Non-bilayer structure formation -Decrease dimerization ATP synthase in the cristae -Reduce OXPHOS |
-Synthetic liposomes -Drosophila |
[28,69] |
| -RCs (I, III) -RSCs (I, III, IV) |
-Reduction in RCs -Misfold of RSCs |
-Mouse | [70] |
| ADP/ATP carrier (AAC) | -Destabilized protein structure -OXPHOS defects. |
-Yeast -Human cell lines |
[39] |
| MCU, MICU1 | -Reduced mitochondrial Ca2+ uptake -Inactivation of pyruvate dehydrogenase |
-BTHS patient cells and cardiac tissue | [41,42] |
| PARL | -Increase the expression of PARL leading to apoptosis | HEK293 cells | [71] |
| DRP1 | -Reduce mitochondrial fission | -Synthetic liposomes | [47] |
| p-ser579-DRP1 p-ser600-DRP1 |
-Reduce DRP1 activation | -Synthetic liposomes | [72] |
| OPA1 | -Alterations mitochondrial fusion | -Synthetic liposomes | [48] |
| LC3 | -Decrease mitophagy | -Synthetic liposomes -SH-SY5Y cells |
[67] |
| Cytochrome c | -Apoptosis | -HL-60 cells | [73] |
Abbreviations: BTHS, Barth syndrome; CL, cardiolipin; MIC, mitochondrial contact site and cristae organizing system; MUC, mitochondrial calcium uniporter; MICU, mitochondrial calcium uptake 1; RSCs; respiratory supercomplexes; RCs respiratory complexes; OXPHOS, oxidative phosphorylation; PARL, presenilin-associated rhomboid-like protein; DRP1; dynamin-related protein 1; OPA1, optic atrophy 1; LC3, microtubule-associated protein 1A/1B-light chain 3.