Table 1.

Summarization of key mitochondrial regulators during male germ cell development

Germ cell types	Name	Mitochondrial location	Functions	Phenotypes in mutant mice	References
Primordial germ cell (PGC)	αKG	Matrix	Mouse ESC self-renewal, differentiation of human ESCs, PGC fate enhancement	Not available	[40]
	mitosRNA	Matrix	Synchronization and nuclear-mitochondrial communication	Not available	[45]
Prospermatogonia (gonocytes)	TDRD1	IMC	piRNA biogenesis	Male infertile, female fertile	[48, 49]
	MITOPLD	IMC, mitochondria	Primary piRNA processing	Male infertile, female fertile	[53, 54]
	GASZ	IMC, mitochondria	piRNA metabolism	Male infertile, female fertile	[55, 66]
Spermatogonia	MFN1	OMM	Mitochondrial fusion	Male infertile	[62, 63, 65, 66]
	MFN2	OMM, MAM	Mitochondrial fusion	Male infertile	[62, 63, 65]
	SHP2	Mitochondria	Cell growth and development, tissue inflammation, and cellular chemotaxis	Male infertile	[69, 70]
Spermatocyte	EF4	OMM	Quality-control in translation	Male infertile	[74]
	PHB	IMM	Respiratory chain subunits assembly, mitochondrial biogenesis, and mitophagy	Male infertile	[81]
	TFAM	Matrix	mtDNA copy number	Male infertile	[83]
	PHGPx	Mitochondria	antioxidative defense	Male infertile	[91]
	GPAT2	Mitochondria	de novo synthesis of triacylglycerol and glycerophospholipids	Male infertile, female fertile	[92, 96]
Spermatid and spermatozoon	TAZ	IMM	Tetrapalmitoyl-CL (TPCL) formation	Male infertile	[101]
	ANT4	Mitochondria	Exchange ADP and ATP across lipid bilayers	Male infertile	[101, 114, 115]
	ARMC12	OMM	Mitochondrial sheath formation	Male infertile	[123]
	GK2	OMM	Glycerol metabolism, mitochondrial sheath formation	Male infertile	[124, 125]
	TBC1D21	OMM	Mitochondrial sheath formation	Male infertile	[123, 126]
	FIS1	OMM	Mitochondrial fission and mitophagy	Male infertile	[129]
	MFF	OMM	Mitochondrial sheath formation	Male sub-infertile	[131]

αKG, α-ketoglutarate; ESC, embryonic stem cell; TDRD1, Tudor domain-containing 1/Mouse Tudor repeat-1; IMC, inter-mitochondrial cement; MitoPLD, phospholipase D family member 6; OMM, outer mitochondrial membrane; MAM, mitochondrial-associated ER membrane; IMM, inner mitochondrial membrane; MFN, The mitochondrial fusion factor mitofusins; EF4, elongation factor 4; PHB, prohibitin; TFAM, mitochondrial transcription factor A; PHGPx: phospholipid hydroperoxide glutathione peroxidase; GPAT2, glycerol-3-phosphate acyltransferase 2; TAZ, tafazzin; ANT4, ADP/ATP translocase 4; ARMC12, armadillo repeat-containing 12; GK2, glycerin kinase 2; TBC1D21, TBC1 domain family member 21; MFF, mitochondrial fission factor