Table 1 List of genes whose different degree of silencing consistently led to the typical Mit phenotypes.

From: Neuroligin-mediated neurodevelopmental defects are induced by mitochondrial dysfunction and prevented by lutein in C. elegans

Cosmid ID

Gene

Human ortholog, short gene description

Disease

RNAi Power

Phenotype

ZK973.10

lpd-5

NDUFS4, NADH-Dehydrogenase, Fe-S protein complex I subunit

Complex I deficiency (Leigh Syndrome)

Mild

Strong

Few progeny

Slow development; few eggs

Y45G12B.1

nuo-5

NDUFS1, NADH-Ubiquinone Oxidoreductase Fe-S Protein 1; complex I

Complex I deficiency

(Leigh Syndrome)

Mild

Strong

Pale, small, few eggs

Slow development, arrested L2/L3

C09H10.3

nuo-1*

NDUFV1, mitochondrial NADH Dehydrogenase Ubiquinone Flavoprotein 1

Complex I deficiency

(Leigh Syndrome)

Mild

Strong

Pale, thin, sterile

Arrested as L2/L3

T20H4.5

T20H4.5*

NDUFS8, ubiquinone oxidoreductase core subunit S8

Complex I deficiency

(Leigh Syndrome)

Mild

Strong

Pale, small, few eggs

Arrested L2/L3

F53F4.10

F53F4.10

NDUFV2, Fe-S complex I

Complex I deficiency

Parkinson’s Disease Susceptibility

Mild

Strong

Slow development

Slow development, arrested L2/L3

C01F1.2

sco-1

SCO-1, SCO cytochrome c oxidase assembly protein 1

Cytochrome-c oxidase deficiency disease

Mild

Strong

Pale, few progeny

Slow development, sick, thin, sterile

H14A12.2

fum-1

FH, fumarate hydratase, predicted to have fumarate hydratase activity

Fumarase deficiency, Leigh syndrome

Mild

Strong

Few eggs

Few progeny, slow development

T12E12.4

drp-1

DNM1L (dynamin 1 like)

Optic dystrophy

Mild

Strong

Similar to control

Small, pale

D2013.5

eat-3*

OPA-1 or mgm-1, dynamin family GTPase

Dominant optic atrophy

Mild

Strong

Thin

Thin, slow development

F54H12.1

aco-2

ACO2, Mitochondrial aconitase

Infantile cerebellar-retinal degeneration and optic atrophy 9

Mild

Strong

Few progeny

Sterile

Y47G6A.10

spg-7*

AFG3L2, AFG3 like matrix AAA peptidase subunit 2

Spinocerebellar ataxia (SCA28)

Mild

Strong

Pale, small, thin

Slow development, arrested L2

F23B12.5

dlat-1*

DLAT, dihydrolipoamide S-acetyltransferase

pyruvate decarboxylase deficiency

Mild

Strong

Sterile, slow moving

Sterile

K08E3.7

pdr-1*

PARK-2 or parkin, E3 ubiquitin ligase

Parkinson’s disease

Mild

Strong

Slow moving, pale, few progeny

Slow moving, pale, few eggs, protruding vulva

F25B4.6

hmgs-1*

HMGCS1 & HMGCS2, 3-hydroxy-3-methylglutaryl-CoA synthase 1 & 2

Mitochondrial HMG-CoA synthase deficiency

Mild

Strong

Pale, thin, slow moving

Sick, pale, slow moving

C15F1.7

sod-1

SOD-1, Cu-Zn SOD1

Amyotrophic lateral sclerosis

Mild

Strong

Small

Similar to control

T22B11.5

ogdh-1

OGDH, predicted to have oxoglutarate dehydrogenase (succinyl-transferring) activity

Alpha ketoglutarate deficiency

Mild

Strong

Thin, pale, sterile adults

Thin, pale, slow development, sick

W02F12.5

dlst-1*

DLST, dihydrolipoamide S-succinyl transferase

Possible cause of familial Alzheimer’s disease

Mild

Strong

Skinny, pale

Long, pale

Y46G5A.2

cox-10

COX10, complex IV farnesyl transferase

Mitochondrial complex IV deficiency

Mild

Strong

Few eggs, pale

Pale, few eggs

T27E9.1

tag-61*

SLC25A5, solute carrier family 25 member 5, mitochondrial adenine nucleotide transporters

Mitochondrial phosphate carrier deficiency

Mild

Strong

Thin, pale

Small, pale, sterile, protruding vulva

F101.12

aldo-2

ALDOB, encodes a fructose-bisphosphate aldolase

Hereditary fructose intolerance

Mild

Strong

Pale

Sick, pale

  1. Underlined genes are complex I subunits giving a typical Mit mutants phenotype in our RNAi screen, which we mainly followed up on in this study.
  2. *These clones showed a strong phenotype already in the parental generation (P0, see Fig. 1) when used undiluted, and the mild effect was thus achieved by diluting the dsRNA expressing bacteria (P0, 1/10 or 1/15). Lifespan for these clones were carried out accordingly in the parental generation (P0) left undiluted or diluted (Supplementary Table 2).
  3. For all the other clones a mild phenotype was observed in the undiluted parental generation (P0), while a strong one in the first filial generation (F1). Accordingly, lifespan with these clones were completed with P0 & F1 on undiluted dsRNA expressing bacteria (Supplementary Data 1). Nonetheless, since clones T20H4.5, eat-3 and spg-7 displayed a significant phenotype (but not as strong as others) already in the parental generation, their lifespan was assessed in P0/F1 as well as in diluted/undiluted conditions (see Supplementary Table 2 and Supplementary Data 1).