superpathway of L-citrulline metabolism

Pathway network for the superpathway of L-citrulline metabolism SuperPath

Sources:

PubChem
WikiPathways
Reactome

Pathways in the superpathway of L-citrulline metabolism SuperPath

#	Name	Source	Genes
1	superpathway of L-citrulline metabolism	PubChem	ALDH18A1 ARG1 ARG2 ASL ASS1 CPS1 NOS1 NOS2 NOS3 OAT OTC PRODH (see all 12) (see less)
2	Urea cycle and associated pathways	WikiPathways	ALDH4A1 ARG1 ASL ASS1 CPS1 FH GLS2 GLUD1 GOT1 GOT2 GPT LDHB MDH1 MDH2 NAGS NOS1 NOS2 NOS3 OAT OTC PYCR1 SLC25A12 SLC25A13 SLC25A15 (see all 24) (see less)
3	Urea cycle and metabolism of amino groups	WikiPathways	ACY1 ALDH18A1 ARG1 ARG2 ASL ASS1 CKB CKM CPS1 GAMT GATM GLUD1 NAGS OAT ODC1 OTC PYCR1 PYCR3 SARDH SMS SRM (see all 21) (see less)
4	Urea cycle	Reactome	ARG1 ARG2 ASL ASS1 CPS1 NAGS NMRAL1 OTC SLC25A15 SLC25A2 (see all 10) (see less)
5	Effects of nitric oxide	WikiPathways	HBA1 MB MT-CO1 NOS1 NOS2 NOS3 XDH (see all 7) (see less)
6	urea cycle	PubChem	ARG1 ARG2 ASL ASS1 CPS1 OTC (see all 6) (see less)
7	L-citrulline biosynthesis	PubChem	ALDH18A1 ARG1 ARG2 OAT OTC PRODH (see all 6) (see less)
8	nitric oxide biosynthesis II (mammals)	PubChem	ASL ASS1 NOS1 NOS2 NOS3
9	citrulline-nitric oxide cycle	PubChem	ASL ASS1 NOS1 NOS2 NOS3
10	Nitric oxide metabolism in cystic fibrosis	WikiPathways	NOS2 NOS3

Gene overlap in member pathways for superpathway of L-citrulline metabolism SuperPath

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

#	Symbol	Description	Category	# Related Pathways
1	ASS1	Argininosuccinate Synthase 1	Protein Coding	7
2	ASL	Argininosuccinate Lyase	Protein Coding	7
3	ARG1	Arginase 1	Protein Coding	6
4	OTC	Ornithine Transcarbamylase	Protein Coding	6
5	NOS3	Nitric Oxide Synthase 3	Protein Coding	6
6	NOS2	Nitric Oxide Synthase 2	Protein Coding	6
7	NOS1	Nitric Oxide Synthase 1	Protein Coding	5
8	ARG2	Arginase 2	Protein Coding	5
9	CPS1	Carbamoyl-Phosphate Synthase 1	Protein Coding	5
10	OAT	Ornithine Aminotransferase	Protein Coding	4
11	ALDH18A1	Aldehyde Dehydrogenase 18 Family Member A1	Protein Coding	3
12	NAGS	N-Acetylglutamate Synthase	Protein Coding	3
13	PRODH	Proline Dehydrogenase 1	Protein Coding	2
14	SLC25A15	Solute Carrier Family 25 Member 15	Protein Coding	2
15	GLUD1	Glutamate Dehydrogenase 1	Protein Coding	2
16	PYCR1	Pyrroline-5-Carboxylate Reductase 1	Protein Coding	2
17	GLS2	Glutaminase 2	Protein Coding	1
18	ALDH4A1	Aldehyde Dehydrogenase 4 Family Member A1	Protein Coding	1
19	SLC25A13	Solute Carrier Family 25 Member 13	Protein Coding	1
20	SLC25A12	Solute Carrier Family 25 Member 12	Protein Coding	1
21	GOT1	Glutamic-Oxaloacetic Transaminase 1	Protein Coding	1
22	GOT2	Glutamic-Oxaloacetic Transaminase 2	Protein Coding	1
23	MDH2	Malate Dehydrogenase 2	Protein Coding	1
24	GPT	Glutamic--Pyruvic Transaminase	Protein Coding	1
25	LDHB	Lactate Dehydrogenase B	Protein Coding	1
26	FH	Fumarate Hydratase	Protein Coding	1
27	MDH1	Malate Dehydrogenase 1	Protein Coding	1
28	SRM	Spermidine Synthase	Protein Coding	1
29	SMS	Spermine Synthase	Protein Coding	1
30	PYCR3	Pyrroline-5-Carboxylate Reductase 3	Protein Coding	1
31	ACY1	Aminoacylase 1	Protein Coding	1
32	SARDH	Sarcosine Dehydrogenase	Protein Coding	1
33	ODC1	Ornithine Decarboxylase 1	Protein Coding	1
34	GATM	Glycine Amidinotransferase	Protein Coding	1
35	CKM	Creatine Kinase, M-Type	Protein Coding	1
36	GAMT	Guanidinoacetate N-Methyltransferase	Protein Coding	1
37	CKB	Creatine Kinase B	Protein Coding	1
38	NMRAL1	NmrA Like Redox Sensor 1	Protein Coding	1
39	SLC25A2	Solute Carrier Family 25 Member 2	Protein Coding	1
40	XDH	Xanthine Dehydrogenase	Protein Coding	1
41	MT-CO1	Mitochondrially Encoded Cytochrome C Oxidase I	Protein Coding	1
42	HBA1	Hemoglobin Subunit Alpha 1	Protein Coding	1
43	MB	Myoglobin	Protein Coding	1

Disorders associated with superpathway of L-citrulline metabolism SuperPath

according to GeneCards Suite gene sharing

#	Disorder	Type	Genes	Score
1	Citrullinemia, classic	Enrichment	ASS1, SLC25A13	5.52
2	Citrullinemia	Enrichment	ASS1, SLC25A13	5.52
3	Citrin deficiency, adolescent or adult onset	Enrichment	ASS1, SLC25A13	5.05
4	Pyloric stenosis, infantile hypertrophic, 1	Enrichment	NOS1	3.43
5	Argininosuccinic aciduria	Enrichment	ASL	3.43
6	Ornithine transcarbamylase deficiency, hyperammonemia due to	Enrichment	OTC	3.35
7	Pulmonary hypertension, neonatal	Enrichment	CPS1	3.35
8	Disorder of ornithine metabolism	Enrichment	OTC	3.35
9	Spastic paraplegia 9a, autosomal dominant	Enrichment	ALDH18A1	3.35
10	Cutis laxa, autosomal recessive, type iiia	Enrichment	ALDH18A1	3.35
11	Spastic paraplegia 9b, autosomal recessive	Enrichment	ALDH18A1	3.35
12	Cutis laxa, autosomal dominant 3	Enrichment	ALDH18A1	3.35
13	Autosomal recessive cutis laxa type iii	Enrichment	ALDH18A1	3.35
14	Choroid disease	Enrichment	OAT	3.35
15	Autosomal dominant complex spastic paraplegia type 9b	Enrichment	ALDH18A1	3.35
16	Myoglobinuria, recurrent	Enrichment	MT-CO1	3.29
17	Myopathy, sarcoplasmic body	Enrichment	MB	3.29
18	Hereditary paraganglioma-pheochromocytoma syndromes	Enrichment	FH, MDH2	3.21
19	Hereditary breast ovarian cancer syndrome	Enrichment	CPS1, SLC25A15	3.15
20	Alzheimer disease 2	Enrichment	NOS3	3.13
21	Pre-eclampsia	Enrichment	NOS3	3.13
22	Hyperornithinemia-hyperammonemia-homocitrullinuria syndrome	Enrichment	SLC25A15	3.13
23	Developmental and epileptic encephalopathy 1	Enrichment	MDH2, SLC25A12	3.06
24	Carbamoyl phosphate synthetase i deficiency, hyperammonemia due to	Enrichment	CPS1	3.05
25	Argininemia	Enrichment	ARG1	3.05
26	Hyperprolinemia, type i	Enrichment	PRODH	3.05
27	Schizophrenia 4	Enrichment	PRODH	3.05
28	Spastic paraplegia 5a, autosomal recessive	Enrichment	ALDH18A1	3.05
29	Xanthinuria, type i	Enrichment	XDH	2.99
30	Methemoglobinemia, alpha type	Enrichment	HBA1	2.99
31	Gyrate atrophy of choroid and retina	Enrichment	OAT	2.88
32	Autosomal dominant cutis laxa	Enrichment	ALDH18A1	2.88
33	Glycogen storage disease ia	Enrichment	ASS1	2.83
34	Idiopathic achalasia	Enrichment	NOS1	2.83
35	N-acetylglutamate synthase deficiency	Enrichment	NAGS	2.83
36	Stroke, ischemic	Enrichment	NOS3	2.83
37	Keratoderma, palmoplantar, with deafness	Enrichment	MT-CO1	2.81
38	Alpha thalassemia-intellectual disability syndrome type 1	Enrichment	HBA1	2.81
39	Xanthinuria, type ii	Enrichment	XDH	2.81
40	Myelodysplastic syndrome with ring sideroblasts	Enrichment	MT-CO1	2.81
41	Cutis laxa, autosomal recessive, type iiib	Enrichment	PYCR1	2.81
42	Bachmann-bupp syndrome	Enrichment	ODC1	2.81
43	Sarcosinemia	Enrichment	SARDH	2.81
44	Intellectual developmental disorder, x-linked, syndromic, snyder-robinson type	Enrichment	SMS	2.81
45	Hyperinsulinemic hypoglycemia, familial, 6	Enrichment	GLUD1	2.81
46	Cutis laxa, autosomal recessive, type iib	Enrichment	PYCR1	2.81
47	Syndromic x-linked intellectual disability snyder type	Enrichment	SMS	2.81
48	Hereditary leiomyomatosis and renal cell cancer	Enrichment	FH	2.75
49	Fumarase deficiency	Enrichment	FH	2.75
50	Developmental and epileptic encephalopathy 39 with leukodystrophy	Enrichment	SLC25A12	2.75
51	Leiomyoma cutis	Enrichment	FH	2.75
52	Aspartate aminotransferase, serum level of, quantitative trait locus 1	Enrichment	GOT1	2.75
53	Lactate dehydrogenase b deficiency	Enrichment	LDHB	2.75
54	Developmental and epileptic encephalopathy 51	Enrichment	MDH2	2.75
55	Fumarate hydratase deficiency	Enrichment	FH	2.75
56	Developmental and epileptic encephalopathy 39	Enrichment	SLC25A12	2.75
57	Citrullinemia type ii	Enrichment	SLC25A13	2.75
58	Erythrocytosis, familial, 7	Enrichment	HBA1	2.69
59	Hemoglobin h disease	Enrichment	HBA1	2.69
60	Hereditary recurrent myoglobinuria	Enrichment	MT-CO1	2.69
61	Alzheimer disease, familial, 1	Enrichment	NOS3	2.60
62	Hypertension, essential	Enrichment	NOS3	2.60
63	Pain disorder	Enrichment	OAT	2.58
64	West syndrome	Enrichment	MDH2, SLC25A12	2.57
65	Deafness, nonsyndromic sensorineural, mitochondrial	Enrichment	MT-CO1	2.51
66	Methemoglobinemia, beta type	Enrichment	HBA1	2.51
67	Autosomal dominant secondary polycythemia	Enrichment	HBA1	2.51
68	Fanconi renotubular syndrome 1	Enrichment	GATM	2.51
69	Cerebral creatine deficiency syndrome 2	Enrichment	GAMT	2.51
70	Cerebral creatine deficiency syndrome	Enrichment	GAMT	2.51
71	Cerebral creatine deficiency syndrome 3	Enrichment	GATM	2.51
72	Creatine deficiency disorders	Enrichment	GAMT	2.51
73	Malaria	Enrichment	NOS2	2.47
74	Leiomyoma, uterine	Enrichment	FH	2.45
75	Hyperprolinemia, type ii	Enrichment	ALDH4A1	2.45
76	Citrin deficiency, neonatal or infantile onset	Enrichment	SLC25A13	2.45
77	Developmental and epileptic encephalopathy 82	Enrichment	GOT2	2.45
78	Developmental and epileptic encephalopathy 88	Enrichment	MDH1	2.45
79	Spinocerebellar ataxia 45	Enrichment	FH	2.45
80	Myasthenic syndrome, congenital, 4c, associated with acetylcholine receptor deficiency	Enrichment	ASL	2.43
81	Heinz body anemias	Enrichment	HBA1	2.38
82	Heinz body anemia	Enrichment	HBA1	2.38
83	Mitochondrial myopathy, infantile, transient	Enrichment	MT-CO1	2.33
84	Alpha-thalassemia	Enrichment	HBA1	2.33
85	Geroderma osteodysplasticum	Enrichment	PYCR1	2.33
86	Fanconi syndrome	Enrichment	GATM	2.33
87	Familial colorectal cancer	Enrichment	MT-CO1	2.29
88	Mitochondrial myopathy with reversible cytochrome c oxidase deficiency	Enrichment	MT-CO1	2.29
89	Rare mitochondrial non-syndromic sensorineural deafness	Enrichment	MT-CO1	2.29
90	Aminoacylase 1 deficiency	Enrichment	ACY1	2.21
91	Primary fanconi renotubular syndrome	Enrichment	GATM	2.21
92	Protein-deficiency anemia	Enrichment	HBA1	2.17
93	Cox deficiency, benign infantile mitochondrial myopathy	Enrichment	MT-CO1	2.14
94	Smith-magenis syndrome	Enrichment	SMS	2.11
95	Primary ovarian insufficiency	Enrichment	NOS3	2.06
96	Hyperinsulinemic hypoglycemia, familial, 1	Enrichment	GLUD1	2.03
97	Nonsyndromic genetic hyperinsulinism	Enrichment	GLUD1	2.03
98	Mitochondrial complex iv deficiency, nuclear type 1	Enrichment	MT-CO1	1.95
99	Congenital nervous system abnormality	Enrichment	ALDH18A1, GAMT	1.94
100	Nervous system disease	Enrichment	ALDH18A1, GAMT	1.94
101	Tetralogy of fallot	Enrichment	MT-CO1	1.87
102	Hydrops fetalis, nonimmune	Enrichment	HBA1	1.87
103	Mitochondrial complex v deficiency, mitochondrial type 1	Enrichment	MT-CO1	1.87
104	Neuropathy, ataxia, and retinitis pigmentosa	Enrichment	MT-CO1	1.87
105	Striatonigral degeneration, infantile, mitochondrial	Enrichment	MT-CO1	1.87
106	Camptodactyly of fingers	Enrichment	MT-CO1	1.87
107	Cutis laxa	Enrichment	PYCR1	1.73
108	Hereditary spastic paraplegia	Enrichment	ALDH18A1	1.73
109	Leber hereditary optic neuropathy, modifier of	Enrichment	MT-CO1	1.72
110	Optic atrophy plus syndrome	Enrichment	OAT	1.71
111	Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes	Enrichment	MT-CO1	1.71
112	Hypertelorism	Enrichment	MT-CO1	1.58
113	Parkinson's disease	Enrichment	GAMT	1.54
114	Leigh syndrome, nuclear	Enrichment	MT-CO1	1.45
115	Parkinson disease, late-onset	Enrichment	GAMT	1.44
116	Hepatocellular carcinoma	Enrichment	FH	1.42
117	Leigh disease	Enrichment	MT-CO1	1.41
118	Colorectal cancer	Enrichment	MT-CO1	1.36
119	Mitochondrial disease	Enrichment	MT-CO1	1.36
120	Leber plus disease	Enrichment	MT-CO1	1.32
121	Connective tissue disease	Enrichment	PYCR1	1.31
122	Developmental and epileptic encephalopathy	Enrichment	GOT2	1.21
123	Retinitis pigmentosa	Enrichment	MT-CO1	0.98
124	Hereditary retinal dystrophy	Enrichment	OAT	0.91
125	Fundus dystrophy	Enrichment	OAT	0.91
126	Ovarian cancer	Enrichment	FH	0.78
127	Inherited cancer-predisposing syndrome	Enrichment	FH	0.68

¹ WikiPathways

² PubChem

³ R&D Systems

⁴ Reactome

⁵ GeneGo (Thomson Reuters)

⁶ QIAGEN

⁷ PharmGKB

⁸ Cell Signaling Technology

⁹ Sino Biological

¹⁰ Tocris

¹¹ MalaCards

superpathway of L-citrulline metabolism

Pathway Network for superpathway of L-citrulline metabolism

Pathway network for the superpathway of L-citrulline metabolism SuperPath

Member Pathways for superpathway of L-citrulline metabolism

Pathways in the superpathway of L-citrulline metabolism SuperPath

Gene overlap in member pathways for superpathway of L-citrulline metabolism SuperPath

Associated Genes for superpathway of L-citrulline metabolism

Associated Disorders for superpathway of L-citrulline metabolism

Disorders associated with superpathway of L-citrulline metabolism SuperPath

STRING Interaction Network for superpathway of L-citrulline metabolism

Sources for superpathway of L-citrulline metabolism