Kidney development

Pathway network for the Kidney development SuperPath

Sources:

Reactome
WikiPathways

Pathways in the Kidney development SuperPath

#	Name	Source	Genes
1	Kidney development	Reactome	BMP4 CTNNB1 DLL1 EMX2 EYA1 FGF2 FOXC1 FOXC2 GATA3 GDNF GFRA1 GREM1 HNF1B HNF4A HOXA11 HOXA6 HOXB4 HOXC11 HOXD11 ID4 IRX1 IRX2 ITGA8 ITGB1 JAG1 LFNG LHX1 MECOM NPNT OSR1 PAX2 PAX8 PCDH19 PLAC8 POU3F3 RET ROBO2 SALL1 SIX1 SIX2 SLIT2 WFDC2 WNT11 WNT4 WNT9B WT1 (see all 46) (see less)
2	Formation of the ureteric bud	Reactome	BMP4 EYA1 FOXC1 FOXC2 GDNF GFRA1 GREM1 HOXA11 HOXC11 HOXD11 ITGA8 ITGB1 NPNT PAX2 RET ROBO2 SALL1 SIX1 SIX2 SLIT2 WNT11 (see all 21) (see less)
3	Formation of the nephric duct	Reactome	BMP4 CTNNB1 EMX2 FGF2 GATA3 HOXA6 HOXB4 ID4 LHX1 MECOM NPNT OSR1 PAX2 PAX8 PCDH19 PLAC8 RET WFDC2 (see all 18) (see less)
4	GDNF/RET signaling axis	WikiPathways	AGTR2 BMP4 EYA1 FAT4 FOXC1 FOXC2 GATA3 GDNF GREM1 LHX1 PAX2 ROBO2 SALL1 SLIT2 SOX11 SOX17 SPRY1 (see all 17) (see less)
5	Nephron development	Reactome	DLL1 HNF1B HNF4A IRX1 IRX2 JAG1 LFNG LHX1 POU3F3 WNT4 WNT9B WT1 (see all 12) (see less)
6	Formation of intermediate mesoderm	Reactome	BMP4 FGF2 FOXC1 FOXC2 LHX1 OSR1 PAX2 PAX8 (see all 8) (see less)

Gene overlap in member pathways for Kidney development SuperPath

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

#	Symbol	Description	Category	# Related Pathways
1	LHX1	LIM Homeobox 1	Protein Coding	5
2	PAX2	Paired Box 2	Protein Coding	5
3	BMP4	Bone Morphogenetic Protein 4	Protein Coding	5
4	FOXC1	Forkhead Box C1	Protein Coding	4
5	FOXC2	Forkhead Box C2	Protein Coding	4
6	OSR1	Odd-Skipped Related Transcription Factor 1	Protein Coding	3
7	EYA1	EYA Transcriptional Coactivator And Phosphatase 1	Protein Coding	3
8	FGF2	Fibroblast Growth Factor 2	Protein Coding	3
9	NPNT	Nephronectin	Protein Coding	3
10	GATA3	GATA Binding Protein 3	Protein Coding	3
11	GREM1	Gremlin 1, DAN Family BMP Antagonist	Protein Coding	3
12	GDNF	Glial Cell Derived Neurotrophic Factor	Protein Coding	3
13	RET	Ret Proto-Oncogene	Protein Coding	3
14	ROBO2	Roundabout Guidance Receptor 2	Protein Coding	3
15	SALL1	Spalt Like Transcription Factor 1	Protein Coding	3
16	PAX8	Paired Box 8	Protein Coding	3
17	SLIT2	Slit Guidance Ligand 2	Protein Coding	3
18	WFDC2	WAP Four-Disulfide Core Domain 2	Protein Coding	2
19	SIX2	SIX Homeobox 2	Protein Coding	2
20	CTNNB1	Catenin Beta 1	Protein Coding	2
21	IRX2	Iroquois Homeobox 2	Protein Coding	2
22	JAG1	Jagged Canonical Notch Ligand 1	Protein Coding	2
23	EMX2	Empty Spiracles Homeobox 2	Protein Coding	2
24	MECOM	MDS1 And EVI1 Complex Locus	Protein Coding	2
25	GFRA1	GDNF Family Receptor Alpha 1	Protein Coding	2
26	DLL1	Delta Like Canonical Notch Ligand 1	Protein Coding	2
27	HNF4A	Hepatocyte Nuclear Factor 4 Alpha	Protein Coding	2
28	HOXA6	Homeobox A6	Protein Coding	2
29	HOXA11	Homeobox A11	Protein Coding	2
30	HOXB4	Homeobox B4	Protein Coding	2
31	HOXC11	Homeobox C11	Protein Coding	2
32	HOXD11	Homeobox D11	Protein Coding	2
33	ID4	Inhibitor Of DNA Binding 4	Protein Coding	2
34	ITGB1	Integrin Subunit Beta 1	Protein Coding	2
35	LFNG	LFNG O-Fucosylpeptide 3-Beta-N-Acetylglucosaminyltransferase	Protein Coding	2
36	PLAC8	Placenta Associated 8	Protein Coding	2
37	WNT4	Wnt Family Member 4	Protein Coding	2
38	POU3F3	POU Class 3 Homeobox 3	Protein Coding	2
39	PCDH19	Protocadherin 19	Protein Coding	2
40	SIX1	SIX Homeobox 1	Protein Coding	2
41	HNF1B	HNF1 Homeobox B	Protein Coding	2
42	WNT11	Wnt Family Member 11	Protein Coding	2
43	WNT9B	Wnt Family Member 9B	Protein Coding	2
44	WT1	WT1 Transcription Factor	Protein Coding	2
45	IRX1	Iroquois Homeobox 1	Protein Coding	2
46	ITGA8	Integrin Subunit Alpha 8	Protein Coding	2
47	FAT4	FAT Atypical Cadherin 4	Protein Coding	1
48	SOX11	SRY-Box Transcription Factor 11	Protein Coding	1
49	SOX17	SRY-Box Transcription Factor 17	Protein Coding	1
50	AGTR2	Angiotensin II Receptor Type 2	Protein Coding	1
51	SPRY1	Sprouty RTK Signaling Antagonist 1	Protein Coding	1

Disorders associated with Kidney development SuperPath

according to GeneCards Suite gene sharing

#	Disorder	Type	Genes	Score
1	Cakut	Enrichment	FAT4, FOXC1, GATA3, PAX2, SALL1, SLIT2	11.24
2	Renal agenesis, bilateral	Enrichment	EYA1, GFRA1, ITGA8, NPNT, RET, WNT9B	10.93
3	Renal hypodysplasia/aplasia 1	Enrichment	ITGA8, RET, WNT9B	5.69
4	Mullerian duct aplasia, unilateral renal agenesis, and cervicothoracic somite anomalies	Enrichment	HNF1B, WNT4	5.67
5	Chromosome 17q12 deletion syndrome	Enrichment	HNF1B, LHX1	5.67
6	Branchiootic syndrome	Enrichment	EYA1, SIX1	5.64
7	Branchiootic syndrome 1	Enrichment	EYA1, SIX1	5.64
8	Familial vesicoureteral reflux	Enrichment	ROBO2, SOX17	5.35
9	Radio-ulnar synostosis-amegakaryocytic thrombocytopenia syndrome	Enrichment	HOXA11, MECOM	4.95
10	Peters-plus syndrome	Enrichment	BMP4, FOXC1	4.86
11	Branchiootorenal syndrome 1	Enrichment	EYA1, SIX1	4.47
12	Focal segmental glomerulosclerosis	Enrichment	EYA1, PAX2, WT1	4.40
13	Branchiootorenal syndrome	Enrichment	EYA1, SIX1	4.32
14	Congenital central hypoventilation syndrome	Enrichment	GDNF, RET	4.09
15	Renal hypodysplasia/aplasia 3	Enrichment	BMP4, RET	3.96
16	Maturity-onset diabetes of the young	Enrichment	HNF1B, HNF4A	3.87
17	Renal hypoplasia	Enrichment	PAX2, WNT9B	3.78
18	Ovarian cancer	Enrichment	CTNNB1, HNF1B, RET, WT1	3.43
19	Hepatocellular carcinoma	Enrichment	CTNNB1, RET	3.42
20	Hypertelorism and tetralogy of fallot	Enrichment	FOXC1	3.23
21	Microphthalmia, syndromic 6	Enrichment	BMP4	3.23
22	Orofacial cleft 11	Enrichment	BMP4	3.23
23	Pax2-related disorder	Enrichment	PAX2	3.23
24	Type 2 diabetes mellitus	Enrichment	HNF1B, HNF4A	3.22
25	Differentiated thyroid carcinoma	Enrichment	PAX8, RET	3.18
26	Mullerian aplasia and hyperandrogenism	Enrichment	WNT4	3.05
27	Spondylocostal dysostosis 3, autosomal recessive	Enrichment	LFNG	3.05
28	46,xx sex reversal with dysgenesis of kidneys, adrenals, and lungs	Enrichment	WNT4	3.05
29	Prostate cancer, hereditary, 11	Enrichment	HNF1B	3.05
30	Fanconi renotubular syndrome 4 with maturity-onset diabetes of the young	Enrichment	HNF4A	3.05
31	Charcot-marie-tooth disease, axonal, type 2hh	Enrichment	JAG1	3.05
32	Meacham syndrome	Enrichment	WT1	3.05
33	Deafness, congenital heart defects, and posterior embryotoxon	Enrichment	JAG1	3.05
34	Snijders blok-fisher syndrome	Enrichment	POU3F3	3.05
35	Hyperinsulinism due to hnf4a deficiency	Enrichment	HNF4A	3.05
36	Medullary sponge kidney	Enrichment	HNF1B	3.05
37	Renal dysplasia, bilateral	Enrichment	HNF1B	3.05
38	Unilateral multicystic dysplastic kidney	Enrichment	HNF1B	3.05
39	Renal dysplasia, unilateral	Enrichment	HNF1B	3.05
40	Hirschsprung disease 1	Enrichment	GDNF, RET	3.04
41	Papillorenal syndrome	Enrichment	PAX2	2.93
42	Axenfeld-rieger syndrome, type 3	Enrichment	FOXC1	2.93
43	Axenfeld-rieger anomaly with partially absent eye muscles, distinctive face, hydrocephaly, and skeletal abnormalities	Enrichment	FOXC1	2.93
44	Lymphedema-distichiasis syndrome	Enrichment	FOXC2	2.93
45	Anterior segment dysgenesis 3	Enrichment	FOXC1	2.93
46	Focal segmental glomerulosclerosis 7	Enrichment	PAX2	2.93
47	Axenfeld-rieger syndrome	Enrichment	FOXC1	2.93
48	Renal hypoplasia, bilateral	Enrichment	PAX2	2.93
49	Vesicoureteral reflux 2	Enrichment	ROBO2	2.90
50	Townes-brocks syndrome 1	Enrichment	SALL1	2.90
51	Otofaciocervical syndrome 1	Enrichment	EYA1	2.90
52	Vesicoureteral reflux 3	Enrichment	SOX17	2.90
53	Van maldergem syndrome 2	Enrichment	FAT4	2.90
54	Hennekam lymphangiectasia-lymphedema syndrome 2	Enrichment	FAT4	2.90
55	Hirschsprung disease 3	Enrichment	GDNF	2.90
56	Intellectual developmental disorder with microcephaly and with or without ocular malformations or hypogonadotropic hypogonadism	Enrichment	SOX11	2.90
57	Van maldergem syndrome	Enrichment	FAT4	2.90
58	Pulmonary hypertension, primary, 7	Enrichment	SOX17	2.90
59	Multiple endocrine neoplasia, type iib	Enrichment	RET	2.88
60	Radioulnar synostosis with amegakaryocytic thrombocytopenia 2	Enrichment	MECOM	2.88
61	Adenoid ameloblastoma	Enrichment	CTNNB1	2.88
62	Thyroid cancer	Enrichment	RET	2.88
63	Microcystic stromal tumor	Enrichment	CTNNB1	2.88
64	Gastrointestinal system disease	Enrichment	RET	2.88
65	Multiple endocrine neoplasia	Enrichment	RET	2.88
66	Polycystic liver disease	Enrichment	CTNNB1, HNF4A	2.83
67	Autosomal dominant polycystic liver disease	Enrichment	CTNNB1, HNF4A	2.83
68	Renal hypodysplasia/aplasia 4	Enrichment	GFRA1	2.81
69	Deafness, autosomal dominant 23	Enrichment	SIX1	2.81
70	Radioulnar synostosis with amegakaryocytic thrombocytopenia 1	Enrichment	HOXA11	2.81
71	Autosomal dominant nonsyndromic hearing loss 23	Enrichment	SIX1	2.81
72	Six2-related frontonasal dysplasia	Enrichment	SIX2	2.81
73	Tubulointerstitial kidney disease, autosomal dominant 1	Enrichment	HNF1B	2.75
74	Renal cysts and diabetes syndrome	Enrichment	HNF1B	2.75
75	Congenital anomalies of kidney and urinary tract 2	Enrichment	HNF1B	2.75
76	Maturity-onset diabetes of the young, type 1	Enrichment	HNF4A	2.75
77	Denys-drash syndrome	Enrichment	WT1	2.75
78	Nephrotic syndrome, type 4	Enrichment	WT1	2.75
79	Frasier syndrome	Enrichment	WT1	2.75
80	Neurodevelopmental disorder with nonspecific brain abnormalities and with or without seizures	Enrichment	DLL1	2.75
81	Hyperuricemic nephropathy, familial juvenile, 3	Enrichment	HNF1B	2.75
82	Hyperinsulinism	Enrichment	HNF4A	2.75
83	Hyperinsulinism due to hnf1a deficiency	Enrichment	HNF4A	2.75
84	Desmoplastic small round cell tumor	Enrichment	WT1	2.75
85	Hepatoblastoma	Enrichment	CTNNB1, JAG1	2.64
86	Hypoparathyroidism, sensorineural deafness, and renal dysplasia syndrome	Enrichment	GATA3	2.60
87	Van maldergem syndrome 1	Enrichment	FAT4	2.60
88	Townes-brocks syndrome	Enrichment	SALL1	2.60
89	B-lymphoblastic leukemia/lymphoma with hyperdiploidy	Enrichment	GATA3	2.60
90	Hypoparathyroidism-deafness-renal disease syndrome	Enrichment	GATA3	2.60
91	Hereditary mixed polyposis syndrome	Enrichment	GREM1	2.60
92	Otofaciocervical syndrome	Enrichment	EYA1	2.60
93	Autosomal dominant nonsyndromic deafness	Enrichment	GATA3	2.60
94	Alagille syndrome 1	Enrichment	JAG1	2.58
95	Mesothelioma, malignant	Enrichment	WT1	2.58
96	Chromophobe renal cell carcinoma	Enrichment	HNF1B	2.58
97	Developmental and epileptic encephalopathy 9	Enrichment	PCDH19	2.58
98	Osteopathia striata with cranial sclerosis	Enrichment	CTNNB1	2.58
99	Bronchiectasis and nasal polyposis	Enrichment	WFDC2	2.58
100	Childhood hepatocellular carcinoma	Enrichment	CTNNB1	2.58
101	Medullary thyroid carcinoma	Enrichment	RET	2.58
102	Juvenile nasopharyngeal angiofibroma	Enrichment	CTNNB1	2.58
103	Radioulnar synostosis	Enrichment	MECOM	2.58
104	Congenital hypogonadotropic hypogonadism	Enrichment	EMX2	2.58
105	Teratoma	Enrichment	CTNNB1	2.58
106	Acute myeloid leukemia with inv(3)(q21q26.2) or t(3;3)(q21;q26.2)	Enrichment	MECOM	2.58
107	Juvenile glaucoma	Enrichment	FOXC1	2.53
108	Aniridia	Enrichment	FOXC1	2.53
109	Deafness, unilateral	Enrichment	SIX1	2.51
110	Genitourinary tract anomalies	Enrichment	HOXA11	2.51
111	Branchiootic syndrome 3	Enrichment	SIX1	2.51
112	Tetralogy of fallot	Enrichment	JAG1, RET	2.46
113	Anterior segment dysgenesis 5	Enrichment	BMP4	2.45
114	Congenital anomalies of kidney and urinary tract 1	Enrichment	PAX2	2.45
115	Aniridia 1	Enrichment	WT1	2.45
116	Maturity-onset diabetes of the young, type 3	Enrichment	HNF4A	2.45
117	Middle aortic syndrome	Enrichment	JAG1	2.45
118	Hennekam syndrome	Enrichment	FAT4	2.43
119	End stage renal disease	Enrichment	GATA3	2.43
120	Desmoid disease, hereditary	Enrichment	CTNNB1	2.40
121	Thyroid carcinoma, familial medullary	Enrichment	RET	2.40
122	Neurodevelopmental disorder with spastic diplegia and visual defects	Enrichment	CTNNB1	2.40
123	Anus, imperforate	Enrichment	CTNNB1	2.40
124	Exudative vitreoretinopathy 7	Enrichment	CTNNB1	2.40
125	Desmoid tumor	Enrichment	CTNNB1	2.40
126	Gingival overgrowth	Enrichment	RET	2.40
127	Hypothyroidism, congenital, nongoitrous, 2	Enrichment	PAX8	2.38
128	Leber congenital amaurosis 10	Enrichment	WT1	2.35
129	Branchiooculofacial syndrome	Enrichment	EYA1	2.30
130	Polyposis syndrome, hereditary mixed, 1	Enrichment	GREM1	2.30
131	Cystic kidney disease	Enrichment	PAX2	2.28
132	Wilms tumor, aniridia, genitourinary anomalies, and impaired intellectual development syndrome	Enrichment	WT1	2.28
133	Wilms tumor 5	Enrichment	WT1	2.28
134	Renal dysplasia, cystic	Enrichment	WNT9B	2.28
135	Spondylocostal dysostosis, autosomal recessive	Enrichment	LFNG	2.28
136	Kidney clear cell sarcoma	Enrichment	IRX2	2.28
137	Schizencephaly	Enrichment	EMX2	2.28
138	Pilomatrixoma	Enrichment	CTNNB1	2.28
139	Alazami syndrome	Enrichment	CTNNB1	2.28
140	Central hypoventilation syndrome, congenital, 1	Enrichment	RET	2.28
141	Craniopharyngioma	Enrichment	CTNNB1	2.28
142	Haddad syndrome	Enrichment	RET	2.28
143	Colorectal cancer	Enrichment	CTNNB1, RET	2.24
144	Stickler syndrome	Enrichment	BMP4	2.23
145	Genetic steroid-resistant nephrotic syndrome	Enrichment	PAX2, WT1	2.23
146	Exudative vitreoretinopathy 1	Enrichment	CTNNB1	2.18
147	Multiple endocrine neoplasia, type iia	Enrichment	RET	2.18
148	Difference of sex development	Enrichment	WT1	2.15
149	Congenital hypothyroidism	Enrichment	PAX8	2.15
150	Microphthalmia/coloboma 12	Enrichment	PAX2	2.12
151	Leukemia, acute lymphoblastic 3	Enrichment	WT1	2.10
152	Dyskeratosis congenita, autosomal dominant 1	Enrichment	MECOM	2.10
153	Weyers acrofacial dysostosis	Enrichment	CTNNB1	2.10
154	Adrenocortical carcinoma	Enrichment	CTNNB1	2.10
155	Glycine encephalopathy	Enrichment	PCDH19	2.10
156	Sporadic pheochromocytoma/secreting paraganglioma	Enrichment	RET	2.10
157	Cleft lip/palate	Enrichment	BMP4	2.08
158	Coloboma of macula	Enrichment	PAX2	2.05
159	Anterior segment dysgenesis	Enrichment	FOXC1	2.05
160	Dyskeratosis congenita, autosomal dominant 2	Enrichment	MECOM	2.03
161	Gallbladder cancer	Enrichment	CTNNB1	2.03
162	46,xy complete gonadal dysgenesis	Enrichment	WT1	2.01
163	Inherited cancer-predisposing syndrome	Enrichment	MECOM, RET, WT1	2.00
164	Glycine encephalopathy 1	Enrichment	PCDH19	1.98
165	Exudative vitreoretinopathy	Enrichment	CTNNB1	1.98
166	Hypothyroidism	Enrichment	RET	1.98
167	Hypogonadotropic hypogonadism	Enrichment	SOX11	1.95
168	Chronic kidney disease	Enrichment	WNT9B	1.94
169	Adult hepatocellular carcinoma	Enrichment	CTNNB1	1.93
170	46,xy partial gonadal dysgenesis	Enrichment	WT1	1.91
171	Familial colorectal cancer	Enrichment	GREM1	1.90
172	Renal cell carcinoma, nonpapillary	Enrichment	HNF1B	1.88
173	Wilms tumor 1	Enrichment	WT1	1.88
174	Kidney disease	Enrichment	WT1	1.88
175	Septopreoptic holoprosencephaly	Enrichment	DLL1	1.88
176	Midline interhemispheric variant of holoprosencephaly	Enrichment	DLL1	1.88
177	Dravet syndrome	Enrichment	PCDH19	1.88
178	Heritable pulmonary arterial hypertension	Enrichment	SOX17	1.86
179	Microform holoprosencephaly	Enrichment	DLL1	1.85
180	Lobar holoprosencephaly	Enrichment	DLL1	1.85
181	Alobar holoprosencephaly	Enrichment	DLL1	1.83
182	Hydrops fetalis, nonimmune	Enrichment	FOXC2	1.82
183	Heart, malformation of	Enrichment	JAG1	1.80
184	Semilobar holoprosencephaly	Enrichment	DLL1	1.80
185	Pulmonary hypertension, primary, 1	Enrichment	SOX17	1.79
186	Primary ovarian insufficiency	Enrichment	MECOM, WT1	1.77
187	Hypogonadotropic hypogonadism 7 with or without anosmia	Enrichment	SOX11	1.76
188	Non-immune hydrops fetalis	Enrichment	FOXC2	1.74
189	Medulloblastoma	Enrichment	CTNNB1	1.73
190	Pheochromocytoma	Enrichment	RET	1.73
191	Coffin-siris syndrome 1	Enrichment	SOX11	1.73
192	Neural tube defects	Enrichment	ITGB1	1.70
193	Craniosynostosis	Enrichment	SOX11	1.61
194	Nephrotic syndrome	Enrichment	PAX2	1.60
195	Prostate cancer	Enrichment	HNF1B	1.60
196	Hereditary paraganglioma-pheochromocytoma syndromes	Enrichment	RET	1.56
197	Strabismus	Enrichment	PCDH19	1.45
198	Bladder cancer	Enrichment	CTNNB1	1.42
199	Autosomal dominant non-syndromic intellectual disability	Enrichment	DLL1	1.36
200	Non-syndromic x-linked intellectual disability	Enrichment	AGTR2	1.35
201	Epilepsy	Enrichment	PCDH19	1.29
202	Benign epilepsy with centrotemporal spikes	Enrichment	PCDH19	1.28
203	Centralopathic epilepsy	Enrichment	PCDH19	1.26
204	Hereditary breast carcinoma	Enrichment	RET	1.25
205	Sensorineural hearing loss	Enrichment	RET	1.21
206	Thrombocytopenia	Enrichment	MECOM	1.21
207	Hypertelorism	Enrichment	RET	1.17
208	Rare autosomal dominant non-syndromic sensorineural deafness type dfna	Enrichment	SIX1	1.11
209	Rare genetic deafness	Enrichment	EYA1	1.02
210	Breast cancer	Enrichment	RET	1.02
211	Congenital nervous system abnormality	Enrichment	CTNNB1	0.88
212	Nervous system disease	Enrichment	CTNNB1	0.88
213	Microcephaly	Enrichment	CTNNB1	0.82
214	Hereditary retinal dystrophy	Enrichment	PAX2	0.80
215	Fundus dystrophy	Enrichment	PAX2	0.80

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

Kidney development

Pathway Network for Kidney development

Pathway network for the Kidney development SuperPath

Member Pathways for Kidney development

Pathways in the Kidney development SuperPath

Gene overlap in member pathways for Kidney development SuperPath

Associated Genes for Kidney development

Associated Disorders for Kidney development

Disorders associated with Kidney development SuperPath

STRING Interaction Network for Kidney development

Sources for Kidney development