Heparan sulfate/heparin (HS-GAG) metabolism

Pathway network for the Heparan sulfate/heparin (HS-GAG) metabolism SuperPath

Sources:

Reactome

Pathways in the Heparan sulfate/heparin (HS-GAG) metabolism SuperPath

#	Name	Source	Genes
1	Heparan sulfate/heparin (HS-GAG) metabolism	Reactome	AGRN CTSL EXT1 EXT2 EXTL2 EXTL3 GLCE GPC1 GPC2 GPC3 GPC4 GPC5 GPC6 HGSNAT HPSE HPSE2 HS2ST1 HS3ST1 HS3ST2 HS3ST3A1 HS3ST3B1 HS3ST4 HS3ST5 HS3ST6 HS6ST1 HS6ST2 HS6ST3 HSPG2 IDS IDUA NAGLU NDST1 NDST2 NDST3 NDST4 SDC1 SDC2 SDC3 SDC4 SGSH SLC35D2 (see all 41) (see less)
2	HS-GAG biosynthesis	Reactome	AGRN EXT1 EXT2 EXTL2 EXTL3 GLCE GPC1 GPC2 GPC3 GPC4 GPC5 GPC6 HS2ST1 HS3ST1 HS3ST2 HS3ST3A1 HS3ST3B1 HS3ST4 HS3ST5 HS3ST6 HS6ST1 HS6ST2 HS6ST3 HSPG2 NDST1 NDST2 NDST3 NDST4 SDC1 SDC2 SDC3 SDC4 SLC35D2 (see all 33) (see less)
3	HS-GAG degradation	Reactome	AGRN CTSL GPC1 GPC2 GPC3 GPC4 GPC5 GPC6 HGSNAT HPSE HPSE2 HSPG2 IDS IDUA NAGLU SDC1 SDC2 SDC3 SDC4 SGSH (see all 20) (see less)

Gene overlap in member pathways for Heparan sulfate/heparin (HS-GAG) metabolism SuperPath

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

#	Symbol	Description	Category	# Related Pathways
1	GPC6	Glypican 6	Protein Coding	3
2	GPC2	Glypican 2	Protein Coding	3
3	GPC4	Glypican 4	Protein Coding	3
4	GPC5	Glypican 5	Protein Coding	3
5	GPC3	Glypican 3	Protein Coding	3
6	GPC1	Glypican 1	Protein Coding	3
7	HSPG2	Heparan Sulfate Proteoglycan 2	Protein Coding	3
8	AGRN	Agrin	Protein Coding	3
9	SDC1	Syndecan 1	Protein Coding	3
10	SDC2	Syndecan 2	Protein Coding	3
11	SDC4	Syndecan 4	Protein Coding	3
12	SDC3	Syndecan 3	Protein Coding	3
13	HPSE	Heparanase	Protein Coding	2
14	SLC35D2	Solute Carrier Family 35 Member D2	Protein Coding	2
15	HGSNAT	Heparan-Alpha-Glucosaminide N-Acetyltransferase	Protein Coding	2
16	CTSL	Cathepsin L	Protein Coding	2
17	EXT1	Exostosin Glycosyltransferase 1	Protein Coding	2
18	EXT2	Exostosin Glycosyltransferase 2	Protein Coding	2
19	EXTL2	Exostosin Like Glycosyltransferase 2	Protein Coding	2
20	EXTL3	Exostosin Like Glycosyltransferase 3	Protein Coding	2
21	HS3ST5	Heparan Sulfate-Glucosamine 3-Sulfotransferase 5	Protein Coding	2
22	GLCE	Glucuronic Acid Epimerase	Protein Coding	2
23	HS6ST3	Heparan Sulfate 6-O-Sulfotransferase 3	Protein Coding	2
24	NDST1	N-Deacetylase And N-Sulfotransferase 1	Protein Coding	2
25	IDS	Iduronate 2-Sulfatase	Protein Coding	2
26	IDUA	Alpha-L-Iduronidase	Protein Coding	2
27	NAGLU	N-Acetyl-Alpha-Glucosaminidase	Protein Coding	2
28	HPSE2	Heparanase 2 (Inactive)	Protein Coding	2
29	SGSH	N-Sulfoglucosamine Sulfohydrolase	Protein Coding	2
30	NDST4	N-Deacetylase And N-Sulfotransferase 4	Protein Coding	2
31	HS3ST6	Heparan Sulfate-Glucosamine 3-Sulfotransferase 6	Protein Coding	2
32	NDST2	N-Deacetylase And N-Sulfotransferase 2	Protein Coding	2
33	HS6ST2	Heparan Sulfate 6-O-Sulfotransferase 2	Protein Coding	2
34	NDST3	N-Deacetylase And N-Sulfotransferase 3	Protein Coding	2
35	HS6ST1	Heparan Sulfate 6-O-Sulfotransferase 1	Protein Coding	2
36	HS2ST1	Heparan Sulfate 2-O-Sulfotransferase 1	Protein Coding	2
37	HS3ST4	Heparan Sulfate-Glucosamine 3-Sulfotransferase 4	Protein Coding	2
38	HS3ST3B1	Heparan Sulfate-Glucosamine 3-Sulfotransferase 3B1	Protein Coding	2
39	HS3ST3A1	Heparan Sulfate-Glucosamine 3-Sulfotransferase 3A1	Protein Coding	2
40	HS3ST2	Heparan Sulfate-Glucosamine 3-Sulfotransferase 2	Protein Coding	2
41	HS3ST1	Heparan Sulfate-Glucosamine 3-Sulfotransferase 1	Protein Coding	2

Disorders associated with Heparan sulfate/heparin (HS-GAG) metabolism SuperPath

according to GeneCards Suite gene sharing

#	Disorder	Type	Genes	Score
1	Mucopolysaccharidosis iii	Enrichment	HGSNAT, NAGLU, SGSH	7.26
2	Exostoses, multiple, type i	Enrichment	EXT1, EXT2	5.24
3	Simpson-golabi-behmel syndrome, type 1	Enrichment	GPC3, GPC4	5.21
4	Mucopolysaccharidosis, type iiia	Enrichment	IDS, SGSH	4.51
5	Keipert syndrome	Enrichment	GPC4	2.83
6	Chondrosarcoma	Enrichment	EXT1	2.61
7	Neurofacioskeletal syndrome with or without renal agenesis	Enrichment	HS2ST1	2.61
8	Intellectual developmental disorder, autosomal recessive 46	Enrichment	NDST1	2.61
9	Angioedema, hereditary, 8	Enrichment	HS3ST6	2.61
10	Immunoskeletal dysplasia with neurodevelopmental abnormalities	Enrichment	EXTL3	2.61
11	Paganini-miozzo syndrome	Enrichment	HS6ST2	2.61
12	Hypogonadotropic hypogonadism 15 with or without anosmia	Enrichment	HS6ST1	2.61
13	Hereditary multiple osteochondromas	Enrichment	EXT1	2.61
14	Exostosis	Enrichment	EXT1	2.61
15	Arteriosclerosis	Enrichment	HS3ST1	2.61
16	Hereditary multiple exostoses	Enrichment	EXT1	2.61
17	Skeletal dysplasia-t-cell immunodeficiency-developmental delay syndrome	Enrichment	EXTL3	2.61
18	Nephrolithiasis, calcium oxalate, 1	Enrichment	IDUA	2.53
19	Mucopolysaccharidosis, type iiic	Enrichment	HGSNAT	2.53
20	Urofacial syndrome 1	Enrichment	HPSE2	2.53
21	Mucopolysaccharidosis, type iiib	Enrichment	NAGLU	2.53
22	Omodysplasia 1	Enrichment	GPC6	2.53
23	Hurler-scheie syndrome	Enrichment	IDUA	2.53
24	Retinitis pigmentosa 73	Enrichment	HGSNAT	2.53
25	Charcot-marie-tooth disease, axonal, type 2v	Enrichment	NAGLU	2.53
26	Scheie syndrome	Enrichment	IDUA	2.53
27	Schwartz-jampel syndrome, type 1	Enrichment	HSPG2	2.53
28	Hypersulfaturia	Enrichment	IDUA	2.53
29	Calcium oxalate nephrolithiasis	Enrichment	IDUA	2.53
30	Dyssegmental dysplasia, silverman-handmaker type	Enrichment	HSPG2	2.35
31	Mucopolysaccharidosis, type ii	Enrichment	IDS	2.35
32	Hurler syndrome	Enrichment	IDUA	2.35
33	Mucopolysaccharidosis-plus syndrome	Enrichment	IDUA	2.35
34	Mucopolysaccharidosis with skin involvement	Enrichment	IDS	2.35
35	Exostoses, multiple, type ii	Enrichment	EXT2	2.31
36	Seizures, scoliosis, and macrocephaly/microcephaly syndrome	Enrichment	EXT2	2.31
37	Mucopolysaccharidosis, type iva	Enrichment	IDUA	2.13
38	Gitelman syndrome	Enrichment	IDUA	2.13
39	Myasthenic syndrome, congenital, 8	Enrichment	AGRN	2.13
40	Diarrhea	Enrichment	SGSH	2.05
41	Hypertrichosis	Enrichment	NAGLU	2.05
42	Trichorhinophalangeal syndrome, type ii	Enrichment	EXT1	2.01
43	Potocki-shaffer syndrome	Enrichment	EXT2	2.01
44	Coronary artery anomaly	Enrichment	HS3ST1	2.01
45	Hereditary angioedema with normal c1inh not related to f12 or plg variant	Enrichment	HS3ST6	2.01
46	Presynaptic congenital myasthenic syndromes	Enrichment	AGRN	1.79
47	Postsynaptic congenital myasthenic syndromes	Enrichment	AGRN	1.76
48	Chromosome 1p36 deletion syndrome	Enrichment	HSPG2	1.72
49	Congenital myasthenic syndrome	Enrichment	AGRN	1.69
50	Wilms tumor 1	Enrichment	GPC3	1.66
51	Ovarian cancer	Enrichment	EXT1, EXT2	1.60
52	Craniosynostosis	Enrichment	GPC4	1.54
53	Normosmic congenital hypogonadotropic hypogonadism	Enrichment	HS6ST1	1.37
54	Cakut	Enrichment	HPSE2	1.31
55	Hepatoblastoma	Enrichment	EXT2	1.30
56	Kallmann syndrome	Enrichment	HS6ST1	1.26
57	Charcot-marie-tooth disease	Enrichment	NAGLU	1.23
58	Hereditary retinal dystrophy	Enrichment	HGSNAT, SGSH	1.17
59	Fundus dystrophy	Enrichment	HGSNAT, SGSH	1.17
60	Body mass index quantitative trait locus 11	Enrichment	SDC3	1.15
61	Autosomal recessive non-syndromic intellectual disability	Enrichment	NDST1	0.88
62	Complex neurodevelopmental disorder	Enrichment	HS2ST1	0.58
63	Retinitis pigmentosa	Enrichment	HGSNAT	0.57
64	Inherited cancer-predisposing syndrome	Enrichment	EXT2	0.56

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

Heparan sulfate/heparin (HS-GAG) metabolism

Pathway Network for Heparan sulfate/heparin (HS-GAG) metabolism

Pathway network for the Heparan sulfate/heparin (HS-GAG) metabolism SuperPath

Member Pathways for Heparan sulfate/heparin (HS-GAG) metabolism

Pathways in the Heparan sulfate/heparin (HS-GAG) metabolism SuperPath

Gene overlap in member pathways for Heparan sulfate/heparin (HS-GAG) metabolism SuperPath

Associated Genes for Heparan sulfate/heparin (HS-GAG) metabolism

Associated Disorders for Heparan sulfate/heparin (HS-GAG) metabolism

Disorders associated with Heparan sulfate/heparin (HS-GAG) metabolism SuperPath

STRING Interaction Network for Heparan sulfate/heparin (HS-GAG) metabolism

Sources for Heparan sulfate/heparin (HS-GAG) metabolism