rRNA processing in the mitochondrion

Sources:

#	Name	Source	Genes
1	rRNA processing in the mitochondrion	Reactome	ELAC2 FASTKD2 HSD17B10 MRM1 MRM2 MRM3 MTERF3 MTERF4 NGRN NSUN4 PRORP RCC1L RPUSD3 RPUSD4 TFB1M TRMT10C TRUB2 (see all 17) (see less)
2	rRNA modification in the mitochondrion	Reactome	FASTKD2 MRM1 MRM2 MRM3 MTERF3 MTERF4 NGRN NSUN4 RCC1L RPUSD3 RPUSD4 TFB1M TRUB2 (see all 13) (see less)
3	Mitochondrial mRNA modification	Reactome	FASTKD2 LRPPRC MTERF3 MTPAP NGRN RCC1L RPUSD3 RPUSD4 SLIRP TRMT61B TRUB2 (see all 11) (see less)

Ordered by rank within the SuperPath, via the multiplicity of each gene in the constituent pathways

#	Symbol	Description	Category	# Related Pathways
1	FASTKD2	FAST Kinase Domains 2	Protein Coding	3
2	TRUB2	TruB Pseudouridine Synthase Family Member 2	Protein Coding	3
3	RPUSD3	RNA Pseudouridine Synthase D3	Protein Coding	3
4	MTERF3	Mitochondrial Transcription Termination Factor 3	Protein Coding	3
5	NGRN	Neugrin, Neurite Outgrowth Associated	Protein Coding	3
6	RCC1L	RCC1 Like	Protein Coding	3
7	RPUSD4	RNA Pseudouridine Synthase D4	Protein Coding	3
8	MTERF4	Mitochondrial Transcription Termination Factor 4	Protein Coding	2
9	MRM2	Mitochondrial RRNA Methyltransferase 2	Protein Coding	2
10	NSUN4	NOP2/Sun RNA Methyltransferase 4	Protein Coding	2
11	TFB1M	Transcription Factor B1, Mitochondrial	Protein Coding	2
12	MRM3	Mitochondrial RRNA Methyltransferase 3	Protein Coding	2
13	MRM1	Mitochondrial RRNA Methyltransferase 1	Protein Coding	2
14	HSD17B10	Hydroxysteroid 17-Beta Dehydrogenase 10	Protein Coding	1
15	TRMT10C	TRNA Methyltransferase 10C, Mitochondrial RNase P Subunit	Protein Coding	1
16	ELAC2	ElaC Ribonuclease Z 2	Protein Coding	1
17	PRORP	Protein Only RNase P Catalytic Subunit	Protein Coding	1
18	LRPPRC	Leucine Rich Pentatricopeptide Repeat Containing	Protein Coding	1
19	TRMT61B	TRNA Methyltransferase 61B	Protein Coding	1
20	MTPAP	Mitochondrial Poly(A) Polymerase	Protein Coding	1
21	SLIRP	SRA Stem-Loop Interacting RNA Binding Protein	Protein Coding	1

according to GeneCards Suite gene sharing

#	Disorder	Type	Genes	Score
1	Spastic ataxia 4, autosomal recessive	Enrichment	MTPAP	3.09
2	Spastic ataxia 4	Enrichment	MTPAP	3.09
3	Fastkd2-related infantile mitochondrial encephalomyopathy	Enrichment	FASTKD2	3.09
4	Mitochondrial dna depletion syndrome 17	Enrichment	MRM2	3.02
5	Hsd10 mitochondrial disease	Enrichment	HSD17B10	2.90
6	Combined oxidative phosphorylation deficiency 30	Enrichment	TRMT10C	2.90
7	Syndromic x-linked intellectual disability type 10	Enrichment	HSD17B10	2.90
8	Prostate cancer, hereditary, 2	Enrichment	ELAC2	2.90
9	Combined oxidative phosphorylation deficiency 17	Enrichment	ELAC2	2.90
10	Combined oxidative phosphorylation deficiency 44	Enrichment	FASTKD2	2.79
11	Spastic ataxia 10, autosomal recessive	Enrichment	TRUB2	2.79
12	Mitochondrial complex iv deficiency, nuclear type 5	Enrichment	LRPPRC	2.61
13	Coenzyme q10 deficiency, primary, 7	Enrichment	TRUB2	2.61
14	Combined oxidative phosphorylation deficiency 54	Enrichment	PRORP	2.60
15	Mitochondrial encephalomyopathy	Enrichment	SLIRP	2.09
16	Rare mitochondrial non-syndromic sensorineural deafness	Enrichment	TFB1M	2.02
17	Perrault syndrome 2	Enrichment	PRORP	1.95
18	Lactic acidosis	Enrichment	PRORP	1.83
19	Mitochondrial complex iv deficiency, nuclear type 1	Enrichment	FASTKD2	1.75
20	Perrault syndrome 1	Enrichment	PRORP	1.70
21	Prostate cancer	Enrichment	ELAC2	1.45
22	Spastic ataxia	Enrichment	MTPAP	1.38
23	Leigh syndrome, nuclear	Enrichment	FASTKD2	1.26
24	Leigh disease	Enrichment	FASTKD2	1.22
25	Mitochondrial disease	Enrichment	TRMT10C	0.98
26	Ovarian cancer	Enrichment	ELAC2	0.92
27	Microcephaly	Enrichment	PRORP	0.84

¹ WikiPathways

² PubChem

³ R&D Systems

⁴ Reactome

⁵ GeneGo (Thomson Reuters)

⁶ QIAGEN

⁷ PharmGKB

⁹ Sino Biological

¹⁰ Tocris

¹¹ MalaCards

Pathway Network for rRNA processing in the mitochondrion