hu.MAP 3.0: Complex View

Human Protein Complex Map

Complex: huMAP3_10525.1

Complex Portal: CPX-10968

Confidence: Medium High

Genename	Protein Name	Uniprot Annotation Score	Links
CRYZ	Quinone oxidoreductase (EC 1.6.5.5) (NADPH:quinone reductase) (Zeta-crystallin)	5	UniProt NCBI
TRIM43	Tripartite motif-containing protein 43	4	UniProt NCBI
IARS2	Isoleucine--tRNA ligase, mitochondrial (EC 6.1.1.5) (Isoleucyl-tRNA synthetase) (IleRS)	5	UniProt NCBI
NARS2	Asparaginyl-tRNA synthetase (AsnRS) (NARS2) (EC 6.1.1.22) (Asparagine--tRNA ligase, mitochondrial)	5	UniProt NCBI
HSPD1	60 kDa heat shock protein, mitochondrial (EC 5.6.1.7) (60 kDa chaperonin) (Chaperonin 60) (CPN60) (Heat shock protein 60) (HSP-60) (Hsp60) (HuCHA60) (Mitochondrial matrix protein P1) (P60 lymphocyte protein)	5	UniProt NCBI
CHDH	Choline dehydrogenase, mitochondrial (CDH) (CHD) (EC 1.1.99.1)	4	UniProt NCBI
PDPR	Pyruvate dehydrogenase phosphatase regulatory subunit, mitochondrial (PDPr)	3	UniProt NCBI
LARS2	Leucine--tRNA ligase, mitochondrial (EC 6.1.1.4) (Leucyl-tRNA synthetase) (LeuRS)	5	UniProt NCBI
PPOX	Protoporphyrinogen oxidase (PPO) (EC 1.3.3.4)	5	UniProt NCBI
MCCC2	Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial (MCCase subunit beta) (EC 6.4.1.4) (3-methylcrotonyl-CoA carboxylase 2) (3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit) (3-methylcrotonyl-CoA:carbon dioxide ligase subunit beta)	5	UniProt NCBI
TMLHE	Trimethyllysine dioxygenase, mitochondrial (EC 1.14.11.8) (Epsilon-trimethyllysine 2-oxoglutarate dioxygenase) (Epsilon-trimethyllysine hydroxylase) (TML hydroxylase) (TML-alpha-ketoglutarate dioxygenase) (TML dioxygenase) (TMLD)	5	UniProt NCBI
TRMU	Mitochondrial tRNA-specific 2-thiouridylase 1 (EC 2.8.1.14) (MTO2 homolog)	5	UniProt NCBI
MTHFD1L	Monofunctional C1-tetrahydrofolate synthase, mitochondrial (EC 6.3.4.3) (Formyltetrahydrofolate synthetase)	5	UniProt NCBI
MCCC1	Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial (MCCase subunit alpha) (EC 6.4.1.4) (3-methylcrotonyl-CoA carboxylase 1) (3-methylcrotonyl-CoA carboxylase biotin-containing subunit) (3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha)	5	UniProt NCBI
HSD17B8	(3R)-3-hydroxyacyl-CoA dehydrogenase (EC 1.1.1.n12) (17-beta-hydroxysteroid dehydrogenase 8) (17-beta-HSD 8) (HSD17B8) (3-ketoacyl-[acyl-carrier-protein] reductase alpha subunit) (KAR alpha subunit) (3-oxoacyl-[acyl-carrier-protein] reductase) (Estradiol 17-beta-dehydrogenase 8) (EC 1.1.1.62) (Protein Ke6) (Ke6) (Short chain dehydrogenase/reductase family 30C member 1) (Testosterone 17-beta-dehydrogenase 8) (EC 1.1.1.239)	5	UniProt NCBI
ALDH18A1	Delta-1-pyrroline-5-carboxylate synthase (P5CS) (Aldehyde dehydrogenase family 18 member A1) [Includes: Glutamate 5-kinase (GK) (EC 2.7.2.11) (Gamma-glutamyl kinase); Gamma-glutamyl phosphate reductase (GPR) (EC 1.2.1.41) (Glutamate-5-semialdehyde dehydrogenase) (Glutamyl-gamma-semialdehyde dehydrogenase)]	5	UniProt NCBI
MMUT	Methylmalonyl-CoA mutase, mitochondrial (MCM) (EC 5.4.99.2) (Methylmalonyl-CoA isomerase)	5	UniProt NCBI
ACSF3	Malonate--CoA ligase ACSF3, mitochondrial (EC 6.2.1.76) (Acyl-CoA synthetase family member 3)	5	UniProt NCBI
AARS2	Alanine--tRNA ligase, mitochondrial (EC 6.1.1.7) (Alanyl-tRNA synthetase) (AlaRS)	5	UniProt NCBI
FOXRED1	FAD-dependent oxidoreductase domain-containing protein 1 (EC 1.-.-.-)	5	UniProt NCBI
CPS1	Carbamoyl-phosphate synthase [ammonia], mitochondrial (EC 6.3.4.16) (Carbamoyl-phosphate synthetase I) (CPSase I)	5	UniProt NCBI
SIRT3	NAD-dependent protein deacetylase sirtuin-3, mitochondrial (hSIRT3) (EC 2.3.1.286) (Regulatory protein SIR2 homolog 3) (SIR2-like protein 3)	5	UniProt NCBI
CRAT	Carnitine O-acetyltransferase (Carnitine acetylase) (EC 2.3.1.137) (EC 2.3.1.7) (Carnitine acetyltransferase) (CAT) (CrAT)	5	UniProt NCBI
CBR4	3-oxoacyl-[acyl-carrier-protein] reductase (EC 1.1.1.100) (3-ketoacyl-[acyl-carrier-protein] reductase beta subunit) (KAR beta subunit) (Carbonyl reductase family member 4) (CBR4) (Quinone reductase CBR4) (EC 1.6.5.10) (Short chain dehydrogenase/reductase family 45C member 1)	5	UniProt NCBI
CLPX	ATP-dependent Clp protease ATP-binding subunit clpX-like, mitochondrial	5	UniProt NCBI
GFM1	Elongation factor G, mitochondrial (EF-Gmt) (Elongation factor G 1, mitochondrial) (mEF-G 1) (Elongation factor G1) (hEFG1)	5	UniProt NCBI
PPA2	Inorganic pyrophosphatase 2, mitochondrial (EC 3.6.1.1) (Pyrophosphatase SID6-306) (Pyrophosphate phospho-hydrolase 2) (PPase 2)	5	UniProt NCBI
HDHD5	Haloacid dehalogenase-like hydrolase domain-containing 5 (Cat eye syndrome critical region protein 5)	3	UniProt NCBI
ACSF2	Medium-chain acyl-CoA ligase ACSF2, mitochondrial (EC 6.2.1.2)	5	UniProt NCBI
FDXR	NADPH:adrenodoxin oxidoreductase, mitochondrial (AR) (Adrenodoxin reductase) (EC 1.18.1.6) (Ferredoxin--NADP(+) reductase) (Ferredoxin reductase)	5	UniProt NCBI
ATP5MGL	Putative ATP synthase subunit g 2, mitochondrial (ATPase subunit g 2) (ATP synthase membrane subunit g-like protein)	3	UniProt NCBI
DARS2	Aspartate--tRNA ligase, mitochondrial (EC 6.1.1.12) (Aspartyl-tRNA synthetase) (AspRS)	5	UniProt NCBI
PREPL	Prolyl endopeptidase-like (EC 3.4.21.-) (Prolylendopeptidase-like)	5	UniProt NCBI

Enrichments

Term ID	Corrected Pval	Fraction Complex Coverage	Proteins	Term Name
GO:0005739	3.12430778981e-23	0.90625	TRMU SIRT3 DARS2 CBR4 PPOX MCCC1 AARS2 IARS2 CPS1 LARS2 HSD17B8 FOXRED1 MCCC2 ACSF3 ATP5MGL MMUT TMLHE CHDH ACSF2 CRAT CLPX ALDH18A1 MTHFD1L GFM1 HDHD5 FDXR PPA2 PDPR NARS2	mitochondrion
GO:0005759	1.924876613e-17	0.59375	MTHFD1L CLPX LARS2 ACSF3 PDPR PPA2 CBR4 SIRT3 ACSF2 DARS2 MCCC2 GFM1 TMLHE HSD17B8 FDXR MCCC1 CPS1 MMUT IARS2	mitochondrial matrix
HP:0004360	5.9536362239e-13	0.46875	TRMU MCCC2 CPS1 PPA2 DARS2 FOXRED1 CRAT AARS2 LARS2 GFM1 NARS2 PREPL MCCC1 MMUT ACSF3	Abnormality of acid-base homeostasis
GO:0016874	4.21987706382e-12	0.375	MTHFD1L LARS2 ACSF3 CPS1 ACSF2 DARS2 MCCC2 NARS2 ATP5MGL MCCC1 AARS2 IARS2	ligase activity
HP:0001941	8.48849132706e-12	0.4375	TRMU MCCC2 PREPL PPA2 DARS2 FOXRED1 CRAT AARS2 LARS2 GFM1 NARS2 MCCC1 MMUT ACSF3	Acidosis
GO:0003824	3.72262581962e-10	0.9375	PREPL TRIM43 DARS2 CBR4 PPOX MCCC1 AARS2 IARS2 CPS1 LARS2 PDPR FOXRED1 MCCC2 ACSF3 ATP5MGL MMUT TMLHE CRYZ CHDH ACSF2 SIRT3 CRAT NARS2 ALDH18A1 MTHFD1L GFM1 FDXR PPA2 HSD17B8 CLPX	catalytic activity
REAC:R-HSA-379726	1.0771007192e-09	0.1875	IARS2 DARS2 LARS2 PPA2 AARS2 NARS2	Mitochondrial tRNA aminoacylation
REAC:R-HSA-379724	1.00837505929e-07	0.1875	IARS2 DARS2 LARS2 PPA2 AARS2 NARS2	tRNA Aminoacylation
GO:0006520	5.29278816666e-07	0.3125	IARS2 LARS2 DARS2 MMUT MCCC2 NARS2 AARS2 MCCC1 ALDH18A1 CPS1	amino acid metabolic process
HP:0002013	5.38633666243e-07	0.3125	TRMU CPS1 FOXRED1 MMUT MCCC2 NARS2 MCCC1 PPOX ALDH18A1 ACSF3	Vomiting
HP:0001939	1.00723616188e-06	0.625	PREPL TRMU CLPX LARS2 ACSF3 CPS1 PPA2 FDXR FOXRED1 CRAT MMUT DARS2 MCCC2 GFM1 NARS2 MCCC1 AARS2 PPOX ALDH18A1 IARS2	Abnormality of metabolism/homeostasis
KEGG:00970	2.04234932952e-06	0.15625	AARS2 LARS2 DARS2 IARS2 NARS2	Aminoacyl-tRNA biosynthesis
HP:0012337	1.32644548357e-05	0.5	TRMU LARS2 ACSF3 CPS1 PPA2 DARS2 FOXRED1 CRAT AARS2 MCCC2 GFM1 NARS2 PREPL MCCC1 MMUT IARS2	Abnormal homeostasis
HP:0100022	2.2402356019e-05	0.53125	TRMU LARS2 ACSF3 PREPL FDXR FOXRED1 CRAT MMUT DARS2 MCCC2 GFM1 NARS2 MCCC1 AARS2 PPOX ALDH18A1 IARS2	Abnormality of movement
HP:0001942	2.24896617909e-05	0.21875	GFM1 MCCC2 MMUT LARS2 PPA2 MCCC1 AARS2	Metabolic acidosis
HP:0001252	4.7239768231e-05	0.5	TRMU CRAT ACSF3 CPS1 PPA2 DARS2 FOXRED1 MMUT MCCC2 GFM1 NARS2 PREPL FDXR MCCC1 ALDH18A1 IARS2	Hypotonia
HP:0002017	4.80529987883e-05	0.3125	TRMU CPS1 FOXRED1 MMUT MCCC2 NARS2 MCCC1 PPOX ALDH18A1 ACSF3	Nausea and vomiting
HP:0012758	5.0226244827e-05	0.5625	TRMU LARS2 ACSF3 CPS1 FDXR FOXRED1 CRAT MMUT DARS2 MCCC2 GFM1 TMLHE NARS2 PREPL AARS2 MCCC1 ALDH18A1 IARS2	Neurodevelopmental delay
HP:0001992	5.02957209903e-05	0.1875	TRMU MCCC2 ACSF3 MCCC1 PPOX MMUT	Organic aciduria
REAC:R-HSA-1430728	5.50799470242e-05	0.5	HSD17B8 MTHFD1L CRAT ACSF3 PDPR PPA2 CBR4 ACSF2 FDXR MCCC2 TMLHE MCCC1 PPOX CPS1 MMUT CHDH	Metabolism
HP:0003808	6.98198332101e-05	0.53125	TRMU CRAT ACSF3 CPS1 PPA2 FDXR FOXRED1 MMUT DARS2 MCCC2 GFM1 NARS2 PREPL AARS2 MCCC1 ALDH18A1 IARS2	Abnormal muscle tone
KEGG:01100	8.6158859676e-05	0.40625	MTHFD1L CBR4 ACSF3 HSD17B8 SIRT3 MMUT MCCC2 TMLHE PPOX MCCC1 CPS1 ALDH18A1 CHDH	Metabolic pathways
HP:0011804	8.76049249584e-05	0.5625	PREPL TRMU CRAT ACSF3 CPS1 PPA2 FDXR FOXRED1 MMUT DARS2 MCCC2 GFM1 NARS2 MCCC1 AARS2 PPOX ALDH18A1 IARS2	Abnormal muscle physiology
HP:0011015	0.000118904455183	0.25	IARS2 PREPL FOXRED1 MCCC2 ACSF3 MCCC1 MMUT NARS2	Abnormal blood glucose concentration
WP:WP3925	0.000122843983949	0.15625	CPS1 LARS2 MMUT ALDH18A1 MCCC1	Amino acid metabolism
HP:0001250	0.000124699638218	0.5	PREPL ACSF3 LARS2 CPS1 PPA2 DARS2 FOXRED1 MMUT MCCC2 GFM1 TMLHE PPOX FDXR MCCC1 ALDH18A1 NARS2	Seizure
HP:0012072	0.000148646388973	0.1875	TRMU MCCC2 ACSF3 MCCC1 PPOX MMUT	Aciduria
HP:0032943	0.000165698125925	0.1875	TRMU MCCC2 ACSF3 MCCC1 PPOX MMUT	Abnormal urine pH
GO:0043436	0.000170205107858	0.375	MTHFD1L CRAT CBR4 HSD17B8 PPA2 ACSF2 ALDH18A1 MCCC2 ACSF3 MCCC1 MMUT CPS1	oxoacid metabolic process
GO:0006082	0.000176465860247	0.375	MTHFD1L CRAT CBR4 HSD17B8 PPA2 ACSF2 ALDH18A1 MCCC2 ACSF3 MCCC1 MMUT CPS1	organic acid metabolic process
KEGG:00280	0.000179617146429	0.125	ACSF3 MCCC2 MMUT MCCC1	Valine, leucine and isoleucine degradation
REAC:R-HSA-8978868	0.000198740664004	0.1875	HSD17B8 ACSF2 CRAT CBR4 MMUT ACSF3	Fatty acid metabolism
HP:0002151	0.000227688371461	0.21875	TRMU GFM1 DARS2 CRAT LARS2 NARS2 AARS2	Increased serum lactate
HP:0012103	0.000252505679461	0.21875	TRMU PREPL FOXRED1 FDXR GFM1 NARS2 AARS2	Abnormality of the mitochondrion
KEGG:00061	0.000264369125273	0.09375	HSD17B8 ACSF3 CBR4	Fatty acid biosynthesis
HP:0032180	0.000336294314523	0.40625	PREPL TRMU CLPX LARS2 CPS1 FOXRED1 MMUT MCCC2 NARS2 PPOX MCCC1 ALDH18A1 ACSF3	Abnormal circulating metabolite concentration
HP:0031797	0.000504726967634	0.59375	PREPL TRMU CLPX LARS2 CPS1 PPA2 FDXR FOXRED1 CRAT MMUT DARS2 MCCC2 GFM1 NARS2 MCCC1 AARS2 PPOX ALDH18A1 IARS2	Clinical course
HP:0000007	0.000512566490938	0.5625	TRMU LARS2 ACSF3 CPS1 PPA2 FDXR FOXRED1 CRAT MMUT DARS2 MCCC2 GFM1 NARS2 PREPL AARS2 MCCC1 ALDH18A1 IARS2	Autosomal recessive inheritance
GO:0044281	0.000513089483899	0.46875	MTHFD1L CLPX CRAT CBR4 HSD17B8 PPA2 ACSF2 ALDH18A1 MCCC2 ACSF3 ATP5MGL FDXR MCCC1 MMUT CPS1	small molecule metabolic process
HP:0001943	0.00053032168445	0.21875	IARS2 PREPL FOXRED1 MCCC2 ACSF3 MCCC1 MMUT	Hypoglycemia
GO:0016491	0.000534380227698	0.34375	CRYZ CHDH HSD17B8 FOXRED1 FDXR CRAT TMLHE PDPR PPOX ALDH18A1 CBR4	oxidoreductase activity
HP:0012823	0.000568218287187	0.59375	PREPL TRMU CLPX LARS2 CPS1 PPA2 FDXR FOXRED1 CRAT MMUT DARS2 MCCC2 GFM1 NARS2 MCCC1 AARS2 PPOX ALDH18A1 IARS2	Clinical modifier
HP:0003011	0.000574400700392	0.5625	PREPL TRMU CRAT ACSF3 CPS1 PPA2 FDXR FOXRED1 MMUT DARS2 MCCC2 GFM1 NARS2 MCCC1 AARS2 PPOX ALDH18A1 IARS2	Abnormality of the musculature
HP:0001987	0.000628839584699	0.15625	CPS1 MCCC2 MMUT ALDH18A1 MCCC1	Hyperammonemia
CORUM:7190	0.000650497358995	0.0625	MCCC2 MCCC1	3-methylcrotonyl-CoA carboxylase
HP:0012638	0.000669211141242	0.625	PREPL TRMU IARS2 LARS2 ACSF3 CPS1 PPA2 FDXR FOXRED1 CRAT MMUT DARS2 MCCC2 GFM1 TMLHE MCCC1 AARS2 PPOX ALDH18A1 NARS2	Abnormal nervous system physiology
HP:0012759	0.000714752579759	0.5625	TRMU LARS2 ACSF3 CPS1 FDXR FOXRED1 CRAT MMUT DARS2 MCCC2 GFM1 TMLHE NARS2 PREPL AARS2 MCCC1 ALDH18A1 IARS2	Neurodevelopmental abnormality
REAC:R-HSA-72766	0.000717195706135	0.21875	IARS2 GFM1 DARS2 LARS2 PPA2 AARS2 NARS2	Translation
HP:0040156	0.000822089732021	0.15625	ACSF3 PPOX MMUT TRMU MCCC1	Elevated urinary carboxylic acid
GO:0006418	0.000865011844708	0.15625	AARS2 LARS2 DARS2 IARS2 NARS2	tRNA aminoacylation for protein translation
GO:0004812	0.000990690691107	0.15625	AARS2 LARS2 DARS2 IARS2 NARS2	aminoacyl-tRNA ligase activity
GO:0016875	0.000990690691107	0.15625	AARS2 LARS2 DARS2 IARS2 NARS2	ligase activity, forming carbon-oxygen bonds
GO:0043039	0.00128549985792	0.15625	AARS2 LARS2 DARS2 IARS2 NARS2	tRNA aminoacylation
GO:0043038	0.00128549985792	0.15625	AARS2 LARS2 DARS2 IARS2 NARS2	amino acid activation
HP:0003674	0.00143748260755	0.5625	PREPL TRMU CLPX LARS2 CPS1 PPA2 FDXR FOXRED1 CRAT MMUT MCCC2 GFM1 NARS2 MCCC1 AARS2 PPOX ALDH18A1 IARS2	Onset
HP:0000707	0.00159897067892	0.625	PREPL TRMU IARS2 LARS2 ACSF3 CPS1 PPA2 FDXR FOXRED1 CRAT MMUT DARS2 MCCC2 GFM1 TMLHE MCCC1 AARS2 PPOX ALDH18A1 NARS2	Abnormality of the nervous system
HP:0033107	0.00169538217374	0.15625	CPS1 MCCC2 MMUT ALDH18A1 MCCC1	Abnormal circulating proteinogenic amino acid concentration
HP:0011923	0.00169538217374	0.15625	AARS2 GFM1 FOXRED1 NARS2 TRMU	Decreased activity of mitochondrial complex I
GO:0019752	0.00174458009572	0.34375	MTHFD1L CRAT CBR4 HSD17B8 ACSF2 ALDH18A1 MCCC2 ACSF3 MCCC1 MMUT CPS1	carboxylic acid metabolic process
HP:0011446	0.00187671052023	0.5625	PREPL TRMU LARS2 ACSF3 CPS1 FDXR FOXRED1 CRAT MMUT DARS2 MCCC2 GFM1 TMLHE MCCC1 AARS2 PPOX ALDH18A1 NARS2	Abnormality of mental function
HP:0001259	0.001893624611	0.15625	CPS1 PPOX MCCC1 MMUT MCCC2	Coma
WP:WP4686	0.00215673864563	0.09375	MCCC2 MMUT ACSF3	Leucine isoleucine and valine metabolism
HP:0004354	0.00217831994972	0.21875	TRMU MMUT MCCC2 ACSF3 MCCC1 ALDH18A1 CPS1	Abnormal circulating carboxylic acid concentration
REAC:R-HSA-3296482	0.00230569012365	0.09375	MCCC2 MMUT MCCC1	Defects in vitamin and cofactor metabolism
HP:0003287	0.00242704833208	0.1875	TRMU PREPL FOXRED1 GFM1 NARS2 AARS2	Abnormality of mitochondrial metabolism
HP:0034345	0.00281543624432	0.65625	PREPL TRMU IARS2 CLPX LARS2 ACSF3 CPS1 PPA2 FDXR FOXRED1 CRAT MMUT DARS2 MCCC2 GFM1 TMLHE MCCC1 AARS2 PPOX ALDH18A1 NARS2	Mendelian inheritance
HP:0011277	0.00290520305436	0.34375	TRMU MCCC2 PREPL FOXRED1 MMUT LARS2 ACSF3 PPOX MCCC1 ALDH18A1 CPS1	Abnormality of the urinary system physiology
HPA:0570733	0.00297732578532	0.40625	IARS2 GFM1 CBR4 HSD17B8 SIRT3 AARS2 MCCC2 TMLHE FDXR MCCC1 ACSF2 MMUT NARS2	testis; Leydig cells[High]
HP:0000005	0.00348649490151	0.65625	PREPL TRMU IARS2 CLPX LARS2 ACSF3 CPS1 PPA2 FDXR FOXRED1 CRAT MMUT DARS2 MCCC2 GFM1 TMLHE MCCC1 AARS2 PPOX ALDH18A1 NARS2	Mode of inheritance
HP:0033127	0.00419232673746	0.59375	PREPL TRMU LARS2 ACSF3 CPS1 PPA2 FDXR FOXRED1 CRAT MMUT DARS2 MCCC2 GFM1 NARS2 MCCC1 AARS2 PPOX ALDH18A1 IARS2	Abnormality of the musculoskeletal system
GO:0005737	0.00430871972879	1.0	TRMU PREPL TRIM43 DARS2 CBR4 PPOX MCCC1 AARS2 IARS2 CPS1 LARS2 PDPR FOXRED1 MCCC2 ACSF3 ATP5MGL MMUT TMLHE CRYZ CHDH ACSF2 SIRT3 CRAT NARS2 ALDH18A1 MTHFD1L GFM1 HDHD5 FDXR PPA2 HSD17B8 CLPX	cytoplasm
HP:0031980	0.00483967932017	0.15625	ACSF3 PPOX MMUT TRMU MCCC1	Abnormal urine carboxylic acid level
HP:0012443	0.00556712061309	0.5	IARS2 LARS2 CPS1 FDXR FOXRED1 CRAT MMUT DARS2 MCCC2 GFM1 NARS2 PREPL AARS2 MCCC1 ALDH18A1 ACSF3	Abnormal brain morphology
HP:0003110	0.00564996657724	0.28125	TRMU MCCC2 PREPL LARS2 ACSF3 PPOX MCCC1 MMUT CPS1	Abnormality of urine homeostasis
HP:0025032	0.00576168223148	0.46875	PREPL TRMU LARS2 ACSF3 CPS1 FOXRED1 MMUT MCCC2 GFM1 NARS2 MCCC1 AARS2 PPOX ALDH18A1 IARS2	Abnormality of digestive system physiology
HPA:0360052	0.00594616321028	0.625	CRYZ PPA2 IARS2 CLPX LARS2 CBR4 HSD17B8 MTHFD1L SIRT3 CRAT MMUT MCCC2 GFM1 TMLHE AARS2 MCCC1 ACSF2 HDHD5 ALDH18A1 NARS2	parathyroid gland; glandular cells[>=Medium]
HP:0011442	0.00611571351041	0.4375	CPS1 LARS2 FDXR FOXRED1 CRAT MMUT DARS2 MCCC2 GFM1 NARS2 PPOX AARS2 MCCC1 ALDH18A1	Abnormal central motor function
HPA:0100211	0.00616601245986	0.65625	CRYZ PPA2 CHDH CLPX CRAT CBR4 HSD17B8 MTHFD1L DARS2 SIRT3 AARS2 LARS2 GFM1 TMLHE NARS2 PREPL MCCC1 ACSF2 HDHD5 MMUT IARS2	cerebral cortex; neuropil[>=Low]
HP:0011968	0.00683848592276	0.34375	TRMU GFM1 PREPL FOXRED1 ALDH18A1 MCCC2 NARS2 AARS2 MCCC1 MMUT ACSF3	Feeding difficulties
GO:1901566	0.00773914577105	0.4375	MTHFD1L GFM1 ACSF3 CPS1 DARS2 SIRT3 AARS2 LARS2 TMLHE NARS2 ATP5MGL PPOX ALDH18A1 IARS2	organonitrogen compound biosynthetic process
HP:0003215	0.00883525691654	0.125	ACSF3 PPOX TRMU MMUT	Dicarboxylic aciduria
HP:0001270	0.00917951609746	0.3125	IARS2 CRAT PREPL DARS2 ALDH18A1 LARS2 GFM1 FDXR AARS2 TRMU	Motor delay
HP:0000118	0.00993145938579	0.65625	PREPL TRMU IARS2 CLPX LARS2 ACSF3 CPS1 PPA2 FDXR FOXRED1 CRAT MMUT DARS2 MCCC2 GFM1 TMLHE MCCC1 AARS2 PPOX ALDH18A1 NARS2	Phenotypic abnormality
HP:0008972	0.0101749744929	0.15625	AARS2 GFM1 FOXRED1 NARS2 TRMU	Decreased activity of mitochondrial respiratory chain
HP:0033354	0.0106090646579	0.25	TRMU PREPL MCCC2 ACSF3 MCCC1 PPOX MMUT CPS1	Abnormal urine metabolite level
KEGG:01212	0.0108104248256	0.09375	HSD17B8 ACSF3 CBR4	Fatty acid metabolism
HP:0031826	0.0113946340028	0.34375	TRMU CRAT DARS2 AARS2 MCCC2 GFM1 NARS2 MCCC1 PPOX ALDH18A1 IARS2	Abnormal reflex
HP:0011922	0.0118228948843	0.15625	AARS2 GFM1 FOXRED1 NARS2 TRMU	Abnormal activity of mitochondrial respiratory chain
REAC:R-HSA-75105	0.0124577145945	0.09375	HSD17B8 ACSF3 CBR4	Fatty acyl-CoA biosynthesis
HP:0002011	0.0148288645422	0.5	IARS2 LARS2 CPS1 FDXR FOXRED1 CRAT MMUT DARS2 MCCC2 GFM1 NARS2 PREPL AARS2 MCCC1 ALDH18A1 ACSF3	Morphological central nervous system abnormality
HPA:0241102	0.0183268715488	0.59375	CRYZ PPA2 IARS2 CLPX LARS2 CBR4 HSD17B8 MTHFD1L SIRT3 CRAT MMUT MCCC2 GFM1 TMLHE AARS2 MCCC1 ACSF2 ALDH18A1 NARS2	heart muscle; cardiomyocytes[>=Medium]
HPA:0130053	0.0187212445567	0.4375	MTHFD1L GFM1 CBR4 HSD17B8 DARS2 SIRT3 AARS2 MCCC2 NARS2 MCCC1 ACSF2 HDHD5 ALDH18A1 IARS2	colon; glandular cells[High]
REAC:R-HSA-196849	0.0194079216116	0.125	MCCC2 MMUT MTHFD1L MCCC1	Metabolism of water-soluble vitamins and cofactors
HP:0001347	0.0196546971512	0.28125	CRAT DARS2 ALDH18A1 MCCC2 GFM1 NARS2 MCCC1 PPOX AARS2	Hyperreflexia
HP:0003112	0.0206095920555	0.15625	CPS1 MCCC2 MMUT ALDH18A1 MCCC1	Abnormal circulating amino acid concentration
HP:0002157	0.0212922996451	0.15625	CPS1 MCCC2 MMUT ALDH18A1 MCCC1	Azotemia
WP:WP5031	0.021790668163	0.0625	MCCC2 MCCC1	Biotin metabolism including IMDs
HP:0012639	0.0220609886362	0.5	IARS2 LARS2 CPS1 FDXR FOXRED1 CRAT MMUT DARS2 MCCC2 GFM1 NARS2 PREPL AARS2 MCCC1 ALDH18A1 ACSF3	Abnormal nervous system morphology
HP:0001508	0.0227919147951	0.3125	CPS1 PREPL FOXRED1 MMUT MCCC2 ACSF3 FDXR MCCC1 ALDH18A1 AARS2	Failure to thrive
HP:0011458	0.0228371739925	0.40625	PREPL TRMU GFM1 CPS1 FOXRED1 MMUT MCCC2 NARS2 MCCC1 AARS2 PPOX ALDH18A1 ACSF3	Abdominal symptom
HPA:0220053	0.0264256128006	0.46875	CRYZ IARS2 GFM1 HSD17B8 PPA2 DARS2 SIRT3 AARS2 MCCC2 NARS2 MCCC1 ACSF2 HDHD5 ALDH18A1 CBR4	gallbladder; glandular cells[High]
HP:0000119	0.0266818164273	0.46875	PREPL TRMU LARS2 CPS1 FDXR FOXRED1 MMUT DARS2 MCCC2 NARS2 PPOX AARS2 MCCC1 ALDH18A1 ACSF3	Abnormality of the genitourinary system
HP:0004364	0.0291366827326	0.15625	CPS1 MCCC2 MMUT ALDH18A1 MCCC1	Abnormal circulating nitrogen compound concentration
REAC:R-HSA-3371599	0.0293545758858	0.0625	MCCC2 MCCC1	Defective HLCS causes multiple carboxylase deficiency
HP:0011014	0.0343977287522	0.25	IARS2 PREPL FOXRED1 MCCC2 ACSF3 MCCC1 MMUT NARS2	Abnormal glucose homeostasis
HPA:0290402	0.0348591758589	0.53125	CRYZ PPA2 IARS2 CLPX LARS2 CBR4 HSD17B8 MTHFD1L SIRT3 AARS2 MCCC2 GFM1 TMLHE PREPL CPS1 MMUT NARS2	liver; hepatocytes[>=Medium]
HP:0008281	0.0356295932909	0.0625	MCCC2 MCCC1	Acute hyperammonemia
HP:0004911	0.0356295932909	0.0625	MCCC1 MMUT	Episodic metabolic acidosis
HP:0000079	0.0369174426468	0.40625	PREPL TRMU LARS2 CPS1 DARS2 FOXRED1 MMUT MCCC2 NARS2 PPOX MCCC1 ALDH18A1 ACSF3	Abnormality of the urinary system
REAC:R-HSA-3323169	0.0390858987398	0.0625	MCCC2 MCCC1	Defects in biotin (Btn) metabolism
GO:1901363	0.0430849577128	0.3125	CRYZ MTHFD1L HSD17B8 SIRT3 DARS2 CBR4 PPOX MCCC1 MMUT CLPX	heterocyclic compound binding
HP:0003128	0.0487613553875	0.15625	FOXRED1 LARS2 PREPL TRMU AARS2	Lactic acidosis

Edges

Protein 1	Protein 2	Score	ProteomeHD	Interface Overlap
MCCC2	MCCC1	1.0	0.334	structurally_consistent (dimer)
CBR4	HSD17B8	1.0	0.902
HSPD1	ACSF3	0.997	0.158364556962
HSPD1	HDHD5	0.996	0.278
HSD17B8	LARS2	0.995	0.07
IARS2	TMLHE	0.993	0.108
HSPD1	TRMU	0.993	0.316	mutually_exclusive (O14874)
HDHD5	DARS2	0.989	0.19
HSPD1	NARS2	0.987	0.192
HSD17B8	MCCC1	0.986	0.152
MCCC2	TMLHE	0.986	0.248
ACSF2	MTHFD1L	0.985	0.07
HSPD1	TRIM43	0.985
CHDH	LARS2	0.982	0.066
PDPR	TMLHE	0.982	0.124
FDXR	DARS2	0.982	0.076
FOXRED1	DARS2	0.982	0.098
TRIM43	ACSF3	0.982
FOXRED1	MCCC1	0.982	0.028
MCCC1	TRIM43	0.981
LARS2	ACSF2	0.981	0.068	structurally_consistent (Q9H4K7)
CPS1	FOXRED1	0.981	0.062
FDXR	TMLHE	0.981	0.046
IARS2	FDXR	0.981	0.18
PDPR	LARS2	0.981	0.236
IARS2	MCCC2	0.98	0.29
FOXRED1	PPOX	0.98	0.042
PDPR	MTHFD1L	0.98	0.078
HSPD1	TMLHE	0.979	0.13
HSD17B8	PPA2	0.979	0.134
FOXRED1	PDPR	0.979	0.026
CPS1	TMLHE	0.979	0.038
NARS2	DARS2	0.978	0.096
CBR4	CRAT	0.978	0.086
CPS1	FDXR	0.978	0.116
CHDH	NARS2	0.978	0.028
ACSF2	GFM1	0.978	0.11
HSPD1	FOXRED1	0.978	0.308
IARS2	PDPR	0.978	0.284
CHDH	ATP5MGL	0.978
HSD17B8	MTHFD1L	0.977	0.066
LARS2	TRIM43	0.977
PDPR	DARS2	0.977	0.09
IARS2	CLPX	0.977	0.178
MCCC1	TRMU	0.977	0.038
ALDH18A1	CLPX	0.976	0.372
IARS2	TRMU	0.976	0.198
PDPR	MCCC1	0.976	0.142
FOXRED1	MTHFD1L	0.976	0.052
CHDH	PDPR	0.976	0.042
NARS2	MMUT	0.975	0.15
ALDH18A1	NARS2	0.975	0.126
FOXRED1	MCCC2	0.975	0.06
FOXRED1	CLPX	0.974	0.2
PDPR	GFM1	0.974	0.142
FOXRED1	TMLHE	0.973	0.034
NARS2	GFM1	0.972	0.12
CPS1	CLPX	0.972	0.046
PDPR	PPOX	0.972	0.286
FOXRED1	LARS2	0.972	0.144
PDPR	HDHD5	0.972	0.14
PDPR	FDXR	0.972	0.06
CPS1	PDPR	0.971	0.044
IARS2	CRYZ	0.971	0.138
MTHFD1L	TMLHE	0.971	0.064
MCCC1	TMLHE	0.97	0.194
CPS1	MCCC2	0.97	0.042
NARS2	CLPX	0.969	0.11
ACSF2	DARS2	0.969	0.122
PDPR	NARS2	0.969	0.166
HSPD1	CPS1	0.968	0.364
LARS2	CLPX	0.968	0.144
NARS2	MCCC1	0.968	0.17
IARS2	PPOX	0.968	0.178
CPS1	PPOX	0.968	0.068
PDPR	MCCC2	0.968	0.412
IARS2	FOXRED1	0.967	0.104
PDPR	CLPX	0.967	0.064
MCCC2	GFM1	0.967	0.382
CPS1	HDHD5	0.967	0.03
HSPD1	SIRT3	0.967	0.21	structurally_consistent (dimer)
PREPL	LARS2	0.967	0.094
PPOX	LARS2	0.966	0.058
HSD17B8	IARS2	0.966	0.096
NARS2	MTHFD1L	0.966	0.026
NARS2	LARS2	0.966	0.2
ACSF2	TMLHE	0.966	0.144
TMLHE	GFM1	0.965	0.06
HDHD5	TMLHE	0.965	0.04
CBR4	MCCC1	0.964	0.194
CHDH	HDHD5	0.963	0.078
PPOX	DARS2	0.963	0.052
FOXRED1	NARS2	0.962	0.05	structurally_consistent (dimer)
PDPR	MMUT	0.962	0.294
NARS2	SIRT3	0.961	0.184
MTHFD1L	PPA2	0.961	0.082
AARS2	CLPX	0.961	0.322
FDXR	NARS2	0.96	0.0766666666667
HSPD1	GFM1	0.96	0.586
ALDH18A1	ACSF3	0.96	0.1065
CPS1	CHDH	0.96	0.034
NARS2	TMLHE	0.96	0.05
CHDH	GFM1	0.959	0.054
AARS2	CHDH	0.959	0.054
FDXR	MCCC1	0.959	0.128
IARS2	HDHD5	0.958	0.296
MCCC1	DARS2	0.958	0.136
NARS2	HDHD5	0.957	0.126
CHDH	ALDH18A1	0.956	0.092
MTHFD1L	DARS2	0.955	0.07
CBR4	LARS2	0.955	0.09
ALDH18A1	MMUT	0.954	0.10925
IARS2	MTHFD1L	0.954	0.14
ALDH18A1	TMLHE	0.953	0.046
FOXRED1	MMUT	0.953	0.02
PPOX	FDXR	0.952	0.1
PPA2	GFM1	0.951	0.158
IARS2	CHDH	0.951	0.088
CHDH	FOXRED1	0.951	0.038
IARS2	ACSF2	0.951	0.138
FDXR	MTHFD1L	0.95	0.07
CPS1	TRIM43	0.95
ALDH18A1	FOXRED1	0.95	0.308
FOXRED1	HDHD5	0.949	0.16
PREPL	PDPR	0.949	0.04
LARS2	DARS2	0.948	0.144
CHDH	DARS2	0.948	0.05
ALDH18A1	DARS2	0.947	0.224
CLPX	MMUT	0.946	0.058
CPS1	DARS2	0.945	0.058
CPS1	LARS2	0.945	0.046
CHDH	PPOX	0.945	0.07
MCCC2	MTHFD1L	0.944	0.062
ALDH18A1	GFM1	0.944	0.5
IARS2	CPS1	0.944	0.142
CHDH	TMLHE	0.943	0.13
CHDH	MTHFD1L	0.943	0.044
IARS2	PREPL	0.943	0.062
DARS2	GFM1	0.942	0.45
FDXR	GFM1	0.942	0.086
HDHD5	TRMU	0.941	0.222
PPOX	MTHFD1L	0.941	0.034
IARS2	MCCC1	0.941	0.092
HSPD1	DARS2	0.941	0.396
LARS2	HDHD5	0.94	0.162
MTHFD1L	HDHD5	0.94	0.064
TRIM43	MMUT	0.94
DARS2	ACSF3	0.939	0.16
CPS1	ALDH18A1	0.939	0.146
IARS2	LARS2	0.938	0.206
LARS2	TMLHE	0.938	0.106	structurally_consistent (Q9UI43)
MTHFD1L	GFM1	0.937	0.182
CPS1	GFM1	0.937	0.058
IARS2	DARS2	0.937	0.504
CPS1	PPA2	0.936	0.072
NARS2	ACSF3	0.936	0.122
ALDH18A1	LARS2	0.936	0.108
IARS2	ALDH18A1	0.935	0.474
CPS1	MTHFD1L	0.935	0.05
CBR4	CPS1	0.934	0.056
ACSF2	TRIM43	0.934
PPOX	HDHD5	0.934	0.076
ALDH18A1	PDPR	0.933	0.244
AARS2	MTHFD1L	0.932	0.152
TMLHE	DARS2	0.932	0.184
HSPD1	PDPR	0.932	0.152
CLPX	MCCC2	0.93	0.126
IARS2	NARS2	0.93	0.212
IARS2	AARS2	0.929	0.286
MMUT	ACSF3	0.928	0.174
CLPX	GFM1	0.928	0.416
CPS1	NARS2	0.928	0.048
MCCC1	GFM1	0.927	0.126
MCCC2	DARS2	0.926	0.248
PREPL	SIRT3	0.926	0.08
PPOX	TMLHE	0.925	0.084
PPOX	TRMU	0.925	0.058	structurally_consistent (dimer)
AARS2	ALDH18A1	0.925	0.33
CLPX	DARS2	0.924	0.164	structurally_consistent (Q9H0R6)
IARS2	PPA2	0.924	0.26
TMLHE	ACSF3	0.923	0.256
AARS2	PDPR	0.923	0.15
NARS2	MCCC2	0.923	0.156
CBR4	ACSF2	0.922	0.136
PPOX	SIRT3	0.922	0.244
CLPX	MCCC1	0.921	0.036
PPOX	NARS2	0.921	0.114
CPS1	MCCC1	0.921	0.092
HSD17B8	MCCC2	0.921	0.088
CLPX	TMLHE	0.921	0.042
AARS2	ACSF3	0.921	0.182	mutually_exclusive (Q9UI43)
PPOX	TRIM43	0.919		structurally_consistent (dimer)
LARS2	MMUT	0.918	0.16225
MTHFD1L	MMUT	0.918	0.058
AARS2	MCCC2	0.918	0.226
MMUT	DARS2	0.917	0.092
DARS2	SIRT3	0.917	0.124
MCCC2	TRMU	0.916	0.136
LARS2	MCCC2	0.915	0.28
LARS2	MTHFD1L	0.915	0.138
CBR4	MCCC2	0.913	0.17
MCCC1	SIRT3	0.913	0.21
PDPR	PPA2	0.913	0.184
PREPL	GFM1	0.912	0.026
AARS2	CPS1	0.91	0.082
FDXR	HDHD5	0.909	0.066
MTHFD1L	SIRT3	0.908	0.092
HDHD5	PPA2	0.908	0.174
FOXRED1	ACSF2	0.908	0.032
ACSF3	GFM1	0.906	0.102
FOXRED1	GFM1	0.905	0.164
MCCC1	MTHFD1L	0.904	0.04
FDXR	MCCC2	0.903	0.154
CPS1	MMUT	0.901	0.036
CPS1	SIRT3	0.899	0.094
CRAT	MCCC1	0.898	0.084
CRYZ	MMUT	0.897	0.05
CRYZ	GFM1	0.896	0.04
FDXR	LARS2	0.896	0.082
CBR4	MTHFD1L	0.895	0.068
MTHFD1L	ACSF3	0.894	0.05
ALDH18A1	MCCC1	0.893	0.094
CBR4	FDXR	0.893	0.076
PREPL	TMLHE	0.893	0.03
MCCC1	MMUT	0.89	0.23
DARS2	TRMU	0.888	0.038
CLPX	TRIM43	0.888
PREPL	MTHFD1L	0.887	0.03
LARS2	MCCC1	0.887	0.2
ACSF2	PPA2	0.887	0.108
TRIM43	DARS2	0.885
IARS2	GFM1	0.885	0.368
LARS2	GFM1	0.883	0.354
FOXRED1	FDXR	0.883	0.046
CLPX	SIRT3	0.882	0.104
CRYZ	TRIM43	0.882
CPS1	TRMU	0.881	0.102
AARS2	LARS2	0.881	0.234
ALDH18A1	MTHFD1L	0.878	0.55
ACSF2	HDHD5	0.878	0.072
FDXR	TRIM43	0.878
PDPR	ACSF2	0.878	0.076
AARS2	FDXR	0.877	0.118
AARS2	MCCC1	0.873	0.116	mutually_exclusive (Q9UI43)
AARS2	DARS2	0.872	0.186
MCCC2	TRIM43	0.871
PPOX	GFM1	0.869	0.028
ALDH18A1	TRIM43	0.868
MCCC2	ACSF3	0.868	0.25
HSD17B8	ACSF2	0.866	0.07
HSPD1	LARS2	0.865	0.262
TRIM43	SIRT3	0.86
IARS2	SIRT3	0.86	0.368
HSD17B8	ALDH18A1	0.86	0.042
HSPD1	ALDH18A1	0.859	0.64
MMUT	GFM1	0.859	0.23
LARS2	PPA2	0.857	0.17
SIRT3	GFM1	0.857	0.07
FDXR	CLPX	0.856	0.118
CLPX	MTHFD1L	0.853	0.182
ALDH18A1	PPA2	0.852	0.228
MCCC1	ACSF2	0.85	0.18	structurally_consistent (Q9H4K7)
PREPL	MCCC2	0.849	0.012
IARS2	TRIM43	0.848
ALDH18A1	SIRT3	0.848	0.218
CRAT	MCCC2	0.845	0.074
PREPL	DARS2	0.845	0.01
CRYZ	PDPR	0.842	0.092
PPOX	MCCC1	0.841	0.154
NARS2	ACSF2	0.833	0.02
IARS2	HSPD1	0.83	0.498
CPS1	CRAT	0.828	0.06
PREPL	CLPX	0.826	0.074
CHDH	MCCC2	0.826	0.086
NARS2	TRMU	0.826	0.148
CHDH	MMUT	0.825	0.08
PPOX	ACSF2	0.825	0.064
CRYZ	LARS2	0.823	0.044
CBR4	IARS2	0.823	0.314
PREPL	CPS1	0.82	0.046
ALDH18A1	FDXR	0.819	0.188
CPS1	ACSF2	0.819	0.046
AARS2	TMLHE	0.816	0.074
AARS2	NARS2	0.815	0.158
MTHFD1L	TRMU	0.815	0.114
PREPL	MMUT	0.809	0.036
AARS2	FOXRED1	0.809	0.086
LARS2	TRMU	0.808	0.144
ALDH18A1	HDHD5	0.806	0.176
PPA2	DARS2	0.801	0.17
AARS2	MMUT	0.8	0.096
CHDH	FDXR	0.799	0.086
PDPR	SIRT3	0.799	0.25
MCCC1	PPA2	0.798	0.172
CBR4	DARS2	0.796	0.084
TMLHE	TRMU	0.796	0.084
AARS2	PPOX	0.795	0.114
IARS2	MMUT	0.795	0.134
AARS2	GFM1	0.794	0.474
MCCC2	SIRT3	0.791	0.548
PREPL	CRAT	0.791	0.032
HDHD5	SIRT3	0.786	0.174
AARS2	HDHD5	0.785	0.262
CRAT	MTHFD1L	0.784	0.062
AARS2	PREPL	0.782	0.054
CBR4	NARS2	0.782	0.12
ALDH18A1	PPOX	0.781	0.10125
CHDH	MCCC1	0.781	0.068
FOXRED1	TRMU	0.78	0.158
ALDH18A1	ACSF2	0.78	0.142
CHDH	ACSF3	0.778	0.092
HSPD1	MCCC1	0.778	0.142364556962
MCCC2	ACSF2	0.776	0.156
CRAT	TRMU	0.776	0.074
CRYZ	FOXRED1	0.774	0.026
PPOX	MCCC2	0.771	0.112
PREPL	PPA2	0.769	0.018
HDHD5	GFM1	0.761	0.218
PREPL	MCCC1	0.761	0.032
CBR4	FOXRED1	0.759	0.048
CHDH	CLPX	0.755	0.042
PDPR	ACSF3	0.754	0.228
NARS2	CRAT	0.751	0.022
IARS2	ACSF3	0.749	0.208
IARS2	CRAT	0.746	0.16
MCCC2	MMUT	0.744	0.262
HSD17B8	CLPX	0.74	0.054
ACSF3	SIRT3	0.74	0.502
CBR4	PPOX	0.735	0.218
CHDH	SIRT3	0.735	0.114
ACSF2	MMUT	0.734	0.094
HSPD1	MCCC2	0.73	0.35
CRYZ	ALDH18A1	0.726	0.29
MCCC1	HDHD5	0.723	0.084
LARS2	SIRT3	0.718	0.13
PREPL	CHDH	0.718	0.026
PREPL	ALDH18A1	0.717	0.136
PPOX	ACSF3	0.713	0.208
TMLHE	SIRT3	0.708	0.376
TRIM43	HDHD5	0.707
TRMU	SIRT3	0.707	0.068
CLPX	ACSF2	0.704	0.098
CRAT	ACSF3	0.699	0.052
HDHD5	MMUT	0.698	0.068
LARS2	ACSF3	0.697	0.098
MMUT	TMLHE	0.697	0.13
PREPL	ACSF2	0.696	0.016
CRAT	SIRT3	0.694	0.174
MTHFD1L	TRIM43	0.691
FDXR	ACSF2	0.69	0.086
ALDH18A1	CRAT	0.688	0.1
CRYZ	MCCC1	0.681	0.044
CRAT	ACSF2	0.68	0.084
ALDH18A1	TRMU	0.676	0.21
AARS2	HSPD1	0.674	0.418	mutually_exclusive (Q9UI43)
HDHD5	ACSF3	0.674	0.076
CRYZ	FDXR	0.674	0.09
ACSF2	ACSF3	0.666	0.138
CPS1	ACSF3	0.659	0.048
FDXR	CRAT	0.657	0.082
ACSF2	TRMU	0.654	0.046
FDXR	ACSF3	0.651	0.108
CRAT	DARS2	0.65	0.05
CRYZ	MCCC2	0.65	0.144
HSPD1	CHDH	0.648	0.070325
PPOX	CLPX	0.646	0.094
MMUT	PPA2	0.645	0.114
HSPD1	ACSF2	0.636	0.074	structurally_consistent (Q9H4K7)
CRYZ	DARS2	0.625	0.082
PREPL	FDXR	0.623	0.048
LARS2	CRAT	0.62	0.036
CRAT	TMLHE	0.618	0.118
HSD17B8	GFM1	0.615	0.04
TRIM43	TMLHE	0.608
CBR4	TMLHE	0.599	0.134
HSPD1	PPOX	0.598	0.148	mutually_exclusive (Q9UFN0)
MCCC1	ACSF3	0.594	0.298
CRYZ	CPS1	0.584	0.068
MCCC2	HDHD5	0.572	0.222
CRYZ	CHDH	0.572	0.08
CHDH	CRAT	0.57	0.064
PREPL	NARS2	0.567	0.036
CLPX	HDHD5	0.567	0.088
ALDH18A1	MCCC2	0.565	0.256
PDPR	TRMU	0.564	0.138
CRAT	HDHD5	0.542	0.03
HSD17B8	NARS2	0.529	0.022
MCCC2	PPA2	0.529	0.322
CRAT	MMUT	0.528	0.074
CHDH	ACSF2	0.528	0.042
PDPR	CRAT	0.518	0.096
CBR4	HDHD5	0.512	0.078
PPA2	SIRT3	0.51	0.384
AARS2	CRAT	0.507	0.038
CRAT	GFM1	0.501	0.04
FOXRED1	PPA2	0.499	0.038
CRAT	CLPX	0.495	0.046
FOXRED1	TRIM43	0.492
PREPL	PPOX	0.488	0.012
FDXR	SIRT3	0.486	0.126
CBR4	AARS2	0.477	0.082
CLPX	ACSF3	0.477	0.084
CRYZ	ACSF2	0.475	0.164	structurally_consistent (Q9H4K7)
CRYZ	TMLHE	0.472	0.116
TRMU	GFM1	0.462	0.32
FDXR	MMUT	0.438	0.064
HSPD1	MTHFD1L	0.432	0.358
TRMU	ACSF3	0.431	0.062
PDPR	TRIM43	0.429
CRYZ	HDHD5	0.397	0.028
AARS2	ACSF2	0.395	0.108
MMUT	SIRT3	0.392	0.138
HSPD1	CLPX	0.38	0.472
PREPL	ACSF3	0.365	0.038
FOXRED1	SIRT3	0.362	0.044
HSD17B8	FDXR	0.362	0.094
AARS2	SIRT3	0.361	0.08
ACSF2	SIRT3	0.361	0.184
PPOX	CRAT	0.346	0.05
CLPX	TRMU	0.34	0.134
CRYZ	MTHFD1L	0.334	0.054
NARS2	PPA2	0.304	0.132
CRYZ	ACSF3	0.293	0.076
FOXRED1	ACSF3	0.29	0.024
CRYZ	PREPL	0.284	0.026
CLPX	PPA2	0.282	0.112
FDXR	PPA2	0.272	0.098
CRYZ	PPA2	0.264	0.158
CRYZ	CLPX	0.252	0.088
PREPL	FOXRED1	0.25	0.048
TRIM43	GFM1	0.241
FDXR	TRMU	0.23	0.048
CBR4	CRYZ	0.227	0.076
CRYZ	SIRT3	0.219	0.416
FOXRED1	CRAT	0.218	0.052
CBR4	ACSF3	0.215	0.234
CRYZ	CRAT	0.215	0.108
HSPD1	CRAT	0.214	0.11
AARS2	PPA2	0.21	0.132
CHDH	PPA2	0.199	0.06
PPA2	TMLHE	0.181	0.148
CRYZ	PPOX	0.169	0.078
CRAT	PPA2	0.162	0.204
CHDH	TRMU	0.156	0.068
HSD17B8	CPS1	0.15	0.046
AARS2	ATP5MGL	0.147
CRYZ	AARS2	0.142	0.062
CBR4	CHDH	0.139	0.066
HSD17B8	DARS2	0.135	0.074
CBR4	HSPD1	0.132	0.15
AARS2	TRIM43	0.129
PPA2	ACSF3	0.119	0.124
HSPD1	ATP5MGL	0.118
CBR4	SIRT3	0.117	0.43
CBR4	TRIM43	0.117
HSD17B8	AARS2	0.115	0.056
ATP5MGL	MTHFD1L	0.113
HSPD1	FDXR	0.112	0.224
AARS2	TRMU	0.104	0.162
PPA2	TRMU	0.103	0.04
CBR4	GFM1	0.098	0.068
HSD17B8	CRAT	0.096	0.102
CRYZ	NARS2	0.095	0.102
PREPL	TRMU	0.093	0.15
PPOX	MMUT	0.091	0.11
CBR4	CLPX	0.088	0.078
PREPL	HSPD1	0.087	0.21
HSD17B8	TMLHE	0.085	0.084
HSD17B8	PREPL	0.085	0.016
CBR4	MMUT	0.079	0.128
PPOX	PPA2	0.078	0.096
HSD17B8	SIRT3	0.069	0.194
HSD17B8	MMUT	0.067	0.094
HSD17B8	ACSF3	0.064	0.16
CBR4	PDPR	0.062	0.314
CBR4	ALDH18A1	0.059	0.08
TRIM43	PPA2	0.057
CRYZ	HSPD1	0.054	0.384	structurally_consistent (Q9H4K7)
CBR4	PREPL	0.054	0.046
ATP5MGL	CLPX	0.047
HSD17B8	PDPR	0.043	0.162
HSD17B8	HDHD5	0.042	0.044
CBR4	PPA2	0.042	0.128
PREPL	HDHD5	0.035	0.052
HSPD1	MMUT	0.03	0.246
ATP5MGL	GFM1	0.03
PREPL	ATP5MGL	0.026
HSD17B8	FOXRED1	0.025	0.046
HSPD1	PPA2	0.025	0.318
ATP5MGL	ALDH18A1	0.024
CBR4	TRMU	0.022	0.106	mutually_exclusive (O14874)
ATP5MGL	MCCC2	0.021
ATP5MGL	FOXRED1	0.018
IARS2	ATP5MGL	0.017
HSD17B8	PPOX	0.017	0.294
ATP5MGL	DARS2	0.016
ATP5MGL	HDHD5	0.016
MMUT	TRMU	0.014	0.064
ATP5MGL	MMUT	0.011
HSD17B8	CRYZ	0.005	0.08
CRYZ	TRMU	0.002	0.028
HSD17B8	HSPD1	0.002	0.128

Related Complexes

Genename	Complexes
CRYZ	huMAP3_00213.1 huMAP3_05062.1 huMAP3_07052.1 huMAP3_07660.1 huMAP3_09668.1 huMAP3_10334.1 huMAP3_10525.1 huMAP3_13468.1
TRIM43	huMAP3_07677.1 huMAP3_10525.1 huMAP3_11915.1 huMAP3_13468.1
IARS2	huMAP3_00213.1 huMAP3_05062.1 huMAP3_06433.1 huMAP3_07052.1 huMAP3_07660.1 huMAP3_09668.1 huMAP3_10334.1 huMAP3_10525.1 huMAP3_13468.1
NARS2	huMAP3_05734.1 huMAP3_08662.1 huMAP3_10525.1 huMAP3_13468.1
HSPD1	huMAP3_02561.1 huMAP3_05852.1 huMAP3_08924.1 huMAP3_10525.1 huMAP3_13468.1 huMAP3_13755.1
CHDH	huMAP3_03996.1 huMAP3_08856.1 huMAP3_10525.1 huMAP3_13468.1
PDPR	huMAP3_05734.1 huMAP3_07378.1 huMAP3_08617.1 huMAP3_10525.1 huMAP3_13468.1
LARS2	huMAP3_06337.1 huMAP3_09087.1 huMAP3_10525.1 huMAP3_13468.1
PPOX	huMAP3_05734.1 huMAP3_07378.1 huMAP3_08617.1 huMAP3_10525.1 huMAP3_13468.1
MCCC2	huMAP3_06733.1 huMAP3_10525.1 huMAP3_13468.1
TMLHE	huMAP3_09668.1 huMAP3_10525.1 huMAP3_13468.1
TRMU	huMAP3_10525.1 huMAP3_12216.1 huMAP3_13468.1 huMAP3_14126.1
MTHFD1L	huMAP3_07228.1 huMAP3_09854.1 huMAP3_10525.1 huMAP3_13468.1
MCCC1	huMAP3_06733.1 huMAP3_10525.1 huMAP3_13468.1
HSD17B8	huMAP3_00107.1 huMAP3_06905.1 huMAP3_07488.1 huMAP3_07729.1 huMAP3_09087.1 huMAP3_10334.1 huMAP3_10525.1 huMAP3_13468.1
ALDH18A1	huMAP3_04118.1 huMAP3_10525.1 huMAP3_13468.1
MMUT	huMAP3_08662.1 huMAP3_09650.1 huMAP3_10387.1 huMAP3_10525.1 huMAP3_13468.1
ACSF3	huMAP3_10525.1 huMAP3_13468.1
AARS2	huMAP3_01116.1 huMAP3_10525.1 huMAP3_13468.1 huMAP3_14610.1
FOXRED1	huMAP3_05734.1 huMAP3_07378.1 huMAP3_10525.1 huMAP3_13468.1
CPS1	huMAP3_03806.1 huMAP3_09668.1 huMAP3_10525.1 huMAP3_13468.1
SIRT3	huMAP3_10525.1 huMAP3_13468.1
CRAT	huMAP3_06667.1 huMAP3_10178.1 huMAP3_10525.1 huMAP3_13468.1 huMAP3_15291.1
CBR4	huMAP3_00107.1 huMAP3_06667.1 huMAP3_06905.1 huMAP3_09087.1 huMAP3_10178.1 huMAP3_10525.1 huMAP3_13468.1
CLPX	huMAP3_06678.1 huMAP3_08786.1 huMAP3_09668.1 huMAP3_09852.1 huMAP3_10525.1 huMAP3_13468.1 huMAP3_13593.1
GFM1	huMAP3_10525.1 huMAP3_13468.1
PPA2	huMAP3_09624.1 huMAP3_09854.1 huMAP3_10169.1 huMAP3_10525.1 huMAP3_11297.1 huMAP3_13468.1 huMAP3_14442.1
HDHD5	huMAP3_08208.1 huMAP3_08856.1 huMAP3_09668.1 huMAP3_10525.1
ACSF2	huMAP3_07228.1 huMAP3_08696.1 huMAP3_09437.1 huMAP3_10525.1 huMAP3_13468.1
FDXR	huMAP3_05734.1 huMAP3_07378.1 huMAP3_08617.1 huMAP3_10525.1 huMAP3_13468.1
ATP5MGL	huMAP3_03996.1 huMAP3_08856.1 huMAP3_10525.1 huMAP3_11746.1 huMAP3_13468.1 huMAP3_14237.1
DARS2	huMAP3_09668.1 huMAP3_10525.1 huMAP3_13468.1
PREPL	huMAP3_02216.1 huMAP3_06337.1 huMAP3_06831.1 huMAP3_10525.1 huMAP3_13468.1

hu.MAP 3.0: Complex View

Search for a protein

Complex: huMAP3_10525.1

Complex Portal: CPX-10968