FB2026_03 , released September 17, 2026
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Attrill, H. (2024.11.14). Mitochondrial Oxidative Phosphorylation Complex Assembly Factors. 
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FBrf0260880
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Personal communication to FlyBase
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Mitochondrial oxidative phosphorylation (OXPHOS) complex assembly factors are defined as gene products that transiently associate with OXPHOS complex subunits and complex intermediates leading to the assembly of functional holoenzymes (PMID:38086790).
A list of D.mel mitochondrial OXPHOS complex assembly factors was compiled by reviewing potential genes from GO annotation, comparison with human mitochondrial OXPHOS complex assembly factors from HGNC (https://www.genenames.org/data/genegroup/#!/group/645) and Human MitoCarta 3.0 fly orthologs (list supplied by Alex Whitworth, MRC Mitochondrial Biology Unit) and yeast (list supplied by PomBase). PMID:30030361 was the main reference used to guide compilation of the assembly factors.
Respirasome assembly factors are also included. These are factors reported to assist in the assembly of the OXPHOS complexes I,III and IV into a 'supercomplex' (PMID:31919741).
Assembly GO term annotation	FBgn	SYMBOL	NAME	Human Ortholog(s)
mitochondrial respiratory chain complex I assembly	FBgn0086712	Egm	Enigma	ACAD8|ACAD9*|ACADL (ACAD9 is the Mitochondrial Complex I Assembly  complex component)
mitochondrial respiratory chain complex I assembly	FBgn0034578	CG15653	-	COA1
mitochondrial respiratory chain complex I assembly	FBgn0051913	CG31913	-	COA1
mitochondrial respiratory chain complex I assembly	FBgn0037018	Dmac2	Distal membrane arm assembly component 2	DMAC2
mitochondrial respiratory chain complex I assembly	FBgn0028436	ECSIT	ECSIT	ECSIT
mitochondrial respiratory chain complex I assembly	FBgn0033093	CG3270	-	FOXRED1
mitochondrial respiratory chain complex I assembly	FBgn0002021	l(2)37Bb	lethal (2) 37Bb	FOXRED1
mitochondrial respiratory chain complex I assembly	FBgn0259832	CG34229	-	LYRM2
mitochondrial respiratory chain complex I assembly	FBgn0039689	NdufAF1	 NADH:ubiquinone  oxidoreductase complex assembly factor 1	NDUFAF1
mitochondrial respiratory chain complex I assembly	FBgn0263051	CG43346	-	NDUFAF2
mitochondrial respiratory chain complex I assembly	FBgn0034919	NdufAF3	 NADH:ubiquinone  oxidoreductase complex assembly factor 3	NDUFAF3
mitochondrial respiratory chain complex I assembly	FBgn0037777	NdufAF4	 NADH:ubiquinone  oxidoreductase complex assembly factor 4	NDUFAF4
mitochondrial respiratory chain complex I assembly	FBgn0033891	CG8067	-	NDUFAF5
mitochondrial respiratory chain complex I assembly	FBgn0030352	sicily	severe impairment of CI with lengthened youth	NDUFAF6
mitochondrial respiratory chain complex I assembly	FBgn0037880	CG17726	-	NDUFAF7
mitochondrial respiratory chain complex I assembly	FBgn0259726	CG42380	-	NDUFAF8 (only called by eggNOG, Panther)
mitochondrial respiratory chain complex I assembly	FBgn0032986	Nubpl	NUBP iron-sulfur cluster assembly factor, mitochondrial	NUBPL
mitochondrial respiratory chain complex I assembly	FBgn0010340	Timmdc1	Translocase of inner mitochondrial membrane domain containing 1	TIMMDC1
mitochondrial respiratory chain complex I assembly	FBgn0032033	Tmem126	Transmembrane protein 126	TMEM126A|TMEM126B* (TMEM126B is a member of the Mitochondrial Complex I Assembly  complex,  TMEM126A is not but is  involved in the assembly of the ND4 distal membrane module of complex I)
mitochondrial respiratory chain complex I assembly	FBgn0033808	Tmem186	Transmembrane protein 186	TMEM186
mitochondrial respiratory chain complex II assembly	FBgn0033274	CG14757	-	SDHAF2
mitochondrial respiratory chain complex II assembly	FBgn0033523	CG12895	-	SDHAF2
mitochondrial respiratory chain complex II assembly	FBgn0038437	Sdhaf3	Succinate dehydrogenase assembly factor 3	SDHAF3
mitochondrial respiratory chain complex II assembly	FBgn0031971	Sirup	Starvation-upregulated protein	SDHAF4
mitochondrial respiratory chain complex II assembly	FBgn0028844	CG15283	-	SDHAF4
mitochondrial respiratory chain complex III assembly	FBgn0032195	Bcs1	Bcs1 chaperone	BCS1L
mitochondrial respiratory chain complex III assembly	FBgn0050373	CG30373	-	C16orf91
mitochondrial respiratory chain complex III assembly	FBgn0083986	CG34150	-	LYRM7
mitochondrial respiratory chain complex III assembly	FBgn0032744	Ttc19	Tetratricopeptide repeat domain 19	TTC19
mitochondrial respiratory chain complex III assembly	FBgn0035722	Uqcc1	Ubiquinol-cytochrome c reductase complex assembly factor 1	UQCC1
mitochondrial respiratory chain complex III assembly	FBgn0287589	CG46459	-	UQCC2
mitochondrial respiratory chain complex III assembly	FBgn0040715	CG15386	-	UQCC3 (via InterPro; no orthologs found via blastp/DIOPT hit)
mitochondrial respiratory chain complex III assembly	FBgn0052736	sloth1	sloth 1	UQCC5
mitochondrial respiratory chain complex III assembly	FBgn0259204	sloth2	sloth 2	UQCC6
mitochondrial cytochrome c oxidase assembly	FBgn0266708	Cep89	Centrosomal protein 89kDa	CEP89
mitochondrial cytochrome c oxidase assembly	FBgn0040634	CG4186	-	CHCHD7
mitochondrial cytochrome c oxidase assembly	FBgn0032595	CG17996	-	CMC1
mitochondrial cytochrome c oxidase assembly	FBgn0259721	CG42375	-	CMC2
mitochondrial cytochrome c oxidase assembly	FBgn0034578	CG15653	-	COA1
mitochondrial cytochrome c oxidase assembly	FBgn0051913	CG31913	-	COA1
mitochondrial cytochrome c oxidase assembly	FBgn0261353	Ccdc56	Coiled-coil domain containing 56	COA3
mitochondrial cytochrome c oxidase assembly	FBgn0265083	CG44194	-	COA4
mitochondrial cytochrome c oxidase assembly	FBgn0040751	CG13018	-	COA5
mitochondrial cytochrome c oxidase assembly	FBgn0259722	CG42376	-	COA6
mitochondrial cytochrome c oxidase assembly	FBgn0039965	Coa7	Cytochrome c oxidase assembly factor 7	COA7
mitochondrial cytochrome c oxidase assembly	FBgn0051648	Cox11	Cytochrome c oxidase copper chaperone COX11	COX11
mitochondrial cytochrome c oxidase assembly	FBgn0040899	CG17776		COX14
mitochondrial cytochrome c oxidase assembly	FBgn0011476	l(3)neo43	lethal (3) neo43	COX16
mitochondrial cytochrome c oxidase assembly	FBgn0030610	Cox17	Cytochrome c oxidase copper chaperone COX17	COX17
mitochondrial cytochrome c oxidase assembly	FBgn0035960	CG4942	-	COX18
mitochondrial cytochrome c oxidase assembly	FBgn0260222	CG42496	-	COX19
mitochondrial cytochrome c oxidase assembly	FBgn0002354	l(3)87Df	lethal (3) 87Df	COX20
mitochondrial cytochrome c oxidase assembly	FBgn0034248	CG14483	-	PET100
mitochondrial cytochrome c oxidase assembly	FBgn0031247	CG11562	-	PET117
mitochondrial cytochrome c oxidase assembly	FBgn0262467	Scox	Synthesis of cytochrome c oxidase	SCO1/SCO2
mitochondrial cytochrome c oxidase assembly	FBgn0085271	CG34242	-	SMIM20
mitochondrial cytochrome c oxidase assembly	FBgn0029117	Surf1	Surfeit 1	SURF1
mitochondrial cytochrome c oxidase assembly	FBgn0033547	CG12935	-	TMEM223
mitochondrial proton-transporting ATP synthase complex assembly	FBgn0032644	CG5131	-	ATP23
mitochondrial proton-transporting ATP synthase complex assembly	FBgn0022344	CG10340	-	ATPAF1
mitochondrial proton-transporting ATP synthase complex assembly	FBgn0021856	l(2)k14505	lethal (2) k14505	ATPAF2
mitochondrial proton-transporting ATP synthase complex assembly	FBgn0083953	CG34117	-	FMC1
mitochondrial proton-transporting ATP synthase complex assembly	FBgn0030311	CG11699	-	TMEM242
mitochondrial proton-transporting ATP synthase complex assembly	FBgn0035805	Tmem70	Transmembrane protein 70	TMEM70
mitochondrial respirasome assembly	FBgn0037890	CG17734	-	HIGD1A|HIGD1B|HIGD1C
mitochondrial respirasome assembly	FBgn0033519	CG11825	-	HIGD1A|HIGD1C
mitochondrial respirasome assembly	FBgn0030743	CG9921	-	HIGD2A|HIGD2B
mitochondrial respirasome assembly	FBgn0033209	CG12107	-	RAB5IF	
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