Table 2.

The list of drugs targeting mitochondria for the therapeutics of NDDs.

No.	Drug	Application	Target	Mechanism	Ref.
1	MtZFNs	Zebra fishes Hek 293T cells	mtDNA	Bind and cleave the mutated DNA and retains the wild type mtDNA	31
2	Mito TALENs	iPSC cells	mtDNA	Deletion sites for selective cleavage of mtDNA	32
3	Mito CRISPR/Cas9	HEK-292T cells	mtDNA	Mitochondrial specific CAS 9 editing mtDNA m.3243A>G gene sites or knock in homologous recombination strategy to repair mutated mtDNA	33,34
4	DdCBE	HEK293T cells	mtDNA	Specifically catalyzes the transformation of CG base pairs of mtDNA into AT	35
5	Mito-Porter	Hela cells G625A cells	mtRNA	Knockdown the mRNA encoded complex Ⅱ or introduce mito codon regulation system	27
6	ZFNs	iPSC cells	SNCA gene	Reprogram A53T mutation in iPSCs	36
7	TALENs	iPSC cells	CAG gene	Replace pathogenic CAG repeats with normal repeats gene sequences through homologous recombination	37
8	CRISPR/KamiCas9	HD mice model	hHTT-82Q gene	sgHTT1 targeting HTT gene with the aim of permanently blocking leading to HTT silencing	38
9	Bip-V5	Rat 6-OHDA PD model	BAX/BAK	Inhibit BCL-2	39
10	MANF	Rat 6-OHDA PD model	BAX	Inhibit BAX activation	40
11	KuA	Mice MPP+ model	BAX/BCL-2	Reduce BAX/BCL-2 ratio	41
12	VBIT-3, VBIT-4	MEF cells	VDAC1	Inhibition of VDAC1 oligomerization	42
13	VDAC1 N-terminal peptides	NSC-34 cells	VDAC1	Interact with VDAC1, reduce channel conductance	43
14	Nobiletin	Rat model	Complex Ⅰ	Restored the activity of complex Ⅰ	44
15	NaHS	Animal model	ETC	Mediated oxygen consumption rate	45
16	Compound A	Rat PD model	Complex Ⅱ	Blocked BIM-induced apoptosis after BAX is activated on the mitochondria	46
17	rT1	COS-7 cells	ATP synthase	Through TOM20 targets mitochondrial membrane ATP synthase	47
18	Nicotinamide riboside/NR	A-T fibroblasts, Atm–/– mice	NAD+	Boosted NAD+ levels by promoting mitophagy in a PINK1-dependent manner	48
19	Rapamycin	SCIRI mice model	mTOR	Promoted mitophagy and attenuating SCIRI-induced apoptosis	49
20	Urolithin A	C. elegans AD models	PINK1	Stimulated mitophagy, suppressed of neuroinflammation	50
21	Rilmenidine	ALS mice	SOD1	Induced autophagy, promoted autophagic clearance of mutant SOD1 and efficient mitophagy	51
22	Mito Q	Rat LTP model	ROS	Mitochondria-targeted antioxidants	52
23	Mito-TEMPO	Primary microglia	ROS	Inhibit the increase of TNF-α, IL-1β, IL-6 and iNOS	53
24	SS peptides	Caco2 cells	ROS	Targeted delivery of antioxidants to the inner mitochondrial membrane	54
25	Pyridoxine	PC12 cells	GSH	Alleviated the significant decrease of GSH to maintain the activity of complex Ⅰ	55