MCID: NPH018
MIFTS: 56

Nephrogenic Systemic Fibrosis

Categories: Rare diseases, Nephrological diseases, Skin diseases

Aliases & Classifications for Nephrogenic Systemic Fibrosis

MalaCards integrated aliases for Nephrogenic Systemic Fibrosis:

Name: Nephrogenic Systemic Fibrosis 49 55 69
Nephrogenic Fibrosing Dermopathy 49 55 69
Nfd 49
Nsf 49

Classifications:

Orphanet: 55  
Rare skin diseases


External Ids:

Orphanet 55 ORPHA137617
UMLS via Orphanet 70 C1619692 C3888044

Summaries for Nephrogenic Systemic Fibrosis

NIH Rare Diseases : 49 Nephrogenic systemic fibrosis is a condition that affects different parts of the body, particularly the skin. Symptoms of the condition may include progressive swelling and tightening of the skin, sometimes resulting in contractures, and pruritis (itching).  The skin findings are similar to those seen in patients with scleroderma.  Nephrogenic systemic fibrosis was first described in 1997. Being exposed to gadolinium-containing contrast agents during MRI testing has been identified as a significant risk factor for development of this disease. Last updated: 4/24/2013

MalaCards based summary : Nephrogenic Systemic Fibrosis, also known as nephrogenic fibrosing dermopathy, is related to keloids and neuronal intranuclear inclusion disease, and has symptoms including pruritus and exanthema. An important gene associated with Nephrogenic Systemic Fibrosis is TGFB1 (Transforming Growth Factor Beta 1), and among its related pathways/superpathways are ERK Signaling and PEDF Induced Signaling. The drugs Ferrosoferric Oxide and Hematinics have been mentioned in the context of this disorder. Affiliated tissues include skin, kidney and eye, and related phenotypes are cardiovascular system and cellular

Wikipedia : 72 Nephrogenic systemic fibrosis (NSF) or nephrogenic fibrosing dermopathy (NFD) is a rare and serious... more...

Related Diseases for Nephrogenic Systemic Fibrosis

Diseases related to Nephrogenic Systemic Fibrosis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 63)
# Related Disease Score Top Affiliating Genes
1 keloids 29.2 DCN SMAD2 SMAD3 SMAD7 TGFB1
2 neuronal intranuclear inclusion disease 11.2
3 tetanus 11.1
4 fibrosis of extraocular muscles, congenital, 1 11.1
5 infant botulism 10.9
6 epileptic encephalopathy, early infantile, 15 10.9
7 kidney disease 10.3
8 hypochondrogenesis 10.3 SMAD2 SMAD3 SMAD7
9 calcific tendinitis 10.3 EDNRA SPP1 TGFB1
10 pauci-immune glomerulonephritis 10.2 CTGF SMAD2
11 clear cell adenocarcinoma 10.2 SMAD2 SMAD3 SMAD4
12 sialolithiasis 10.2 CTGF SPP1 TGFB1
13 chronic kidney failure 10.1
14 liver disease 10.1
15 perinephritis 10.0 EDN1 EDNRA
16 idiopathic interstitial pneumonia 10.0 CTGF EDN1 TGFB1
17 demyelinating disease 10.0 CCL7 CXCL2 SPP1
18 gingival disease 10.0 CTGF TGFB1 TIMP1
19 mandibulofacial dysostosis with alopecia 10.0 EDN1 EDNRA
20 hypertrophic scars 9.9 SMAD2 SMAD3 TGFB1 TIMP1
21 systemic scleroderma 9.9 CTGF EDN1 TGFB1
22 scleredema 9.9
23 scleromyxedema 9.9
24 sarcoma 9.9
25 inflammatory breast carcinoma 9.9
26 vitreoretinopathy, neovascular inflammatory 9.9 CTGF TGFB1 TIMP1
27 penile disease 9.9 DCN SMAD2 SMAD3 TGFB1
28 urinary tract obstruction 9.8 CTGF PIK3CA SMAD2 SMAD3 TGFB1
29 ureteral disease 9.8 CTGF SMAD2 SMAD3 SMAD7 TGFB1
30 biliary atresia 9.8 CTGF SPP1 TGFB1 TIMP1
31 renal fibrosis 9.8 CTGF SMAD2 SMAD3 SMAD7 TGFB1
32 urinary system disease 9.8 CTGF EDN1 EPO TGFB1
33 interstitial lung disease 9.8 EDN1 TGFB1 TIMP1
34 lymphoma 9.8
35 endotheliitis 9.8
36 respiratory failure 9.8
37 hypercalcemia, infantile, 1 9.8
38 kaposi sarcoma 9.8
39 pure red-cell aplasia 9.8
40 scleroderma, familial progressive 9.8
41 hypothyroidism 9.8
42 arterial calcification, generalized, of infancy, 1 9.8
43 panniculitis 9.8
44 myopathy 9.8
45 histiocytoma 9.8
46 fibrous histiocytoma 9.8
47 diabetes mellitus 9.8
48 calciphylaxis 9.8
49 hepatitis 9.8
50 vasculitis 9.8

Graphical network of the top 20 diseases related to Nephrogenic Systemic Fibrosis:



Diseases related to Nephrogenic Systemic Fibrosis

Symptoms & Phenotypes for Nephrogenic Systemic Fibrosis

UMLS symptoms related to Nephrogenic Systemic Fibrosis:


pruritus, exanthema

MGI Mouse Phenotypes related to Nephrogenic Systemic Fibrosis:

43 (show all 21)
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 10.5 CXCL2 EDNRA CTGF EDN1 SMAD2 F13A1
2 cellular MP:0005384 10.4 DCN EDNRA CTGF EPO PIK3CA MAPKAP1
3 homeostasis/metabolism MP:0005376 10.4 EDN1 DCN EDNRA CTGF PIK3CA EPO
4 growth/size/body region MP:0005378 10.39 EDN1 DCN EDNRA CTGF PIK3CA MAPKAP1
5 endocrine/exocrine gland MP:0005379 10.37 DCN EDNRA CTGF EDN1 SMAD2 F13A1
6 mortality/aging MP:0010768 10.36 DCN EDNRA CTGF EDN1 SMAD2 F13A1
7 embryo MP:0005380 10.31 EDNRA CTGF EDN1 SMAD2 MAPKAP1 PIK3CA
8 hematopoietic system MP:0005397 10.31 DCN EPO F13A1 EDNRA SMAD4 SMAD3
9 immune system MP:0005387 10.29 DCN PIK3CA EPO EDNRA SMAD4 SMAD3
10 craniofacial MP:0005382 10.28 DCN EDNRA CTGF EDN1 SMAD2 SMAD3
11 integument MP:0010771 10.24 DCN EDNRA CTGF EPO SMAD2 PIK3CA
12 digestive/alimentary MP:0005381 10.23 EDN1 DCN CTGF EDNRA SMAD3 SMAD2
13 muscle MP:0005369 10.09 EDN1 DCN PIK3CA EPO EDNRA SMAD4
14 neoplasm MP:0002006 10.06 DCN SMAD2 PIK3CA SMAD3 SMAD4 TGM2
15 liver/biliary system MP:0005370 10.02 CTGF EPO SMAD4 SMAD3 SMAD2 TGFB1
16 normal MP:0002873 9.97 CTGF EDN1 DCN EPO EDNRA SMAD4
17 reproductive system MP:0005389 9.96 DCN PIK3CA F13A1 SMAD4 SMAD3 SMAD2
18 renal/urinary system MP:0005367 9.92 EDN1 DCN EDNRA SMAD4 SMAD3 SPP1
19 respiratory system MP:0005388 9.81 DCN CTGF EPO F13A1 EDNRA SMAD2
20 skeleton MP:0005390 9.73 DCN EDNRA CTGF EDN1 SMAD2 PIK3CA
21 vision/eye MP:0005391 9.23 CTGF DCN PIK3CA MAPKAP1 EDNRA SMAD3

Drugs & Therapeutics for Nephrogenic Systemic Fibrosis

Drugs for Nephrogenic Systemic Fibrosis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 10)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Ferrosoferric Oxide Phase 4,Phase 3,Phase 1
2 Hematinics Phase 4,Phase 3,Phase 1
3 Parenteral Nutrition Solutions Phase 4,Phase 3,Phase 1
4 Pharmaceutical Solutions Phase 4,Phase 3,Phase 1
5 Imatinib Mesylate Phase 3,Phase 2 123596
6 Protein Kinase Inhibitors Phase 3,Phase 2
7 Liver Extracts Phase 3
8 Plasma-lyte 148 Phase 1, Phase 2
9
Iodine Approved, Investigational 7553-56-2 807
10 cadexomer iodine

Interventional clinical trials:

(show all 27)

# Name Status NCT ID Phase Drugs
1 Primovist / Eovist in Renally Impaired Patients Completed NCT00908596 Phase 4 Gadoxetic acid disodium (Primovist, BAY86-4873)
2 Risk of Nephrogenic Systemic Fibrosis (NSF) in Patients With Moderate Renal Insufficiency After the Administration of Magnevist Completed NCT00744939 Phase 4 Gadopentetate dimeglumine (Magnevist, BAY86-4882)
3 Safety of Gadovist in Renally Impaired Patients Completed NCT00828737 Phase 4 Gadobutrol (Gadovist, BAY86-4875)
4 Post-marketing Safety Study in Patients With Moderate Renal Insufficiency Who Receive Omniscan for Contrast-enhanced Magnetic Resonance Imaging (MRI) Completed NCT00908310 Phase 4 Omniscan
5 Ferumoxytol Enhanced Magnetic Resonance Angiography in Chronic Kidney Disease Recruiting NCT02997046 Phase 4
6 Feraheme As An MRI Contrast Agent For Pediatric Congenital Heart Disease Recruiting NCT02752191 Phase 4 ferumoxytol;gadofosveset
7 Plasmapheresis for Nephrogenic Fibrosing Dermopathy (NFD)/Nephrogenic Systemic Fibrosis (NSF) Terminated NCT01078987 Phase 4
8 Nephrogenic Systemic Fibrosis With Gadollinum Unknown status NCT01359345 Phase 2, Phase 3 gadollinum
9 Treatment of Patients With Nephrogenic Systemic Fibrosis With Glivec Completed NCT00981942 Phase 3 Imatinib mesylate (Glivec)
10 Efficacy and Safety of Primovist in Chinese Patients Completed NCT00526188 Phase 3 Gadoxetic Acid Disodium (Primovist, BAY86-4873)
11 Ferumoxytol for Magnetic Resonance Imaging in Patients With Severe Kidney Disease Enrolling by invitation NCT02954510 Phase 3 ferumoxytol
12 Pilot Study of Imatinib Mesylate to Treat Nephrogenic Systemic Fibrosis Completed NCT00677092 Phase 2 Imatinib mesylate
13 The Effect of Crystalloids and Colloids on Visceral Blood Flow Completed NCT01087853 Phase 1, Phase 2 Crystalloid;Colloid
14 MRA With Feraheme in HHT Recruiting NCT02977637 Phase 1
15 Nephrogenic Systemic Fibrosis (NSF): Analysis of Tissue Gadolinium Levels Completed NCT01014754
16 Validation of a Questionnaire to Identify Signs and Symptoms of Nephrogenic Systemic Fibrosis Completed NCT00869479
17 Prospective Evaluation of the Incidence of NSF in Patients With Kidney Disease Undergoing MR Completed NCT01135316 Ablavar
18 Evaluation of the Risk of Nephrogenic Systemic Fibrosis (NSF) in Patients With Chronic Kidney Disease Without Exposure to Gadolinium Based Contrast Agents (GBCA) Completed NCT00773409
19 Evaluation of the Risk of NSF Following ProHance Injection in Patients With Chronic Kidney Disease Completed NCT00600834
20 Evaluation of the Risk of NSF Following MultiHance Injection in Patients With Chronic Kidney Disease Completed NCT00600951
21 Adverse Reactions to MR and CT-examinations (Enhanced and Unenhanced) Completed NCT01132339
22 Magnevist Post-marketing Surveillance in Japan Completed NCT01376739 Gadopentetate dimeglumine (Magnevist, BAY86-4882)
23 Observational Post-marketing Study on the Safety and Efficacy of Dotarem® (SECURE Study) Completed NCT01523873
24 Evaluation of Gd Retention in Human Bone and Skin After MultiHance or ProHance Administration Compared With Control Recruiting NCT03108378
25 Pilot Study of the Effect of Laser on Reversing Chronic Radiation Injury Recruiting NCT01910818
26 Observational Study on the Incidence of NSF in Renal Impaired Patients Following DOTAREM Administration Active, not recruiting NCT01467271
27 Evaluation of Nephrogenic Systemic Fibrosis (NSF) in Patients With Chronic Kidney Disease Following OptiMARK Injection Withdrawn NCT00811863

Search NIH Clinical Center for Nephrogenic Systemic Fibrosis

Genetic Tests for Nephrogenic Systemic Fibrosis

Anatomical Context for Nephrogenic Systemic Fibrosis

MalaCards organs/tissues related to Nephrogenic Systemic Fibrosis:

38
Skin, Kidney, Eye, Testes, Liver, Bone, Heart

Publications for Nephrogenic Systemic Fibrosis

Articles related to Nephrogenic Systemic Fibrosis:

(show top 50) (show all 427)
# Title Authors Year
1
Letter on 'European dermatology forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 2: Scleromyxedema, scleredema and nephrogenic systemic fibrosis'. ( 29283443 )
2018
2
Notice of Withdrawal: MR Imaging and Gadolinium: Reassessing the Risk of Nephrogenic Systemic Fibrosis in Patients with Severe Renal Disease. ( 29261455 )
2018
3
Nephrogenic systemic fibrosis: A survey of the use of gadolinium-based contrast agents in Ghana. ( 28965904 )
2017
4
Gadolinium-Naive Nephrogenic Systemic Fibrosis of Breast Mimicking Inflammatory Breast Carcinoma. ( 28323787 )
2017
5
A unique case of nephrogenic systemic fibrosis from gadolinium exposure in a patient with normal eGFR. ( 29025775 )
2017
6
No Incidence of Nephrogenic Systemic Fibrosis after Gadobenate Dimeglumine Administration in Patients Undergoing Dialysis or Those with Severe Chronic Kidney Disease. ( 28731375 )
2017
7
Photopheresis Provides Significant Long-Lasting Benefit in Nephrogenic Systemic Fibrosis. ( 28695022 )
2017
8
GBCAs and Risk for Nephrogenic Systemic Fibrosis: A Literature Review. ( 28900045 )
2017
9
European dermatology forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 2: Scleromyxedema, scleredema and nephrogenic systemic fibrosis. ( 28786499 )
2017
10
Erythema nodosum masking nephrogenic systemic fibrosis as initial skin manifestation. ( 28738858 )
2017
11
Nephrogenic systemic fibrosis: A 15-year retrospective study at a single tertiary care center. ( 28318680 )
2017
12
Nephrogenic systemic fibrosis. ( 28680623 )
2017
13
Prophylactic Hemodialysis for Protection Against Gadolinium-Induced Nephrogenic Systemic Fibrosis: A Doll's House. ( 28501073 )
2017
14
Nephrogenic systemic fibrosis: a serious adverse reaction to gadolinium - 1997-2006-2016. Part 1. ( 26802069 )
2016
15
Interaction of Gd-DTPA with phosphate and phosphite: toward the reaction intermediate in nephrogenic systemic fibrosis. ( 26906409 )
2016
16
Incidence of Nephrogenic Systemic Fibrosis Using Gadobenate Dimeglumine in 1423 Patients With Renal Insufficiency Compared With Gadodiamide. ( 26885631 )
2016
17
Nephrogenic systemic fibrosis: fibrotic plaques and contracture following exposure to gadolinium-based contrast media. ( 27073153 )
2016
18
Nephrogenic systemic fibrosis: a serious adverse reaction to gadolinium - 1997-2006-2016. Part 2. ( 26802070 )
2016
19
Nephrogenic Systemic Fibrosis Manifesting a Decade After Exposure to Gadolinium. ( 26017458 )
2015
20
Gadobenate Dimeglumine Administration and Nephrogenic Systemic Fibrosis: Is There a Real Risk in Patients with Impaired Renal Function? ( 25875973 )
2015
21
Nephrogenic Systemic Fibrosis Risk After Liver Magnetic Resonance Imaging With Gadoxetate Disodium in Patients With Moderate to Severe Renal Impairment: Results of a Prospective, Open-Label, Multicenter Study. ( 25756684 )
2015
22
Do ASARM peptides play a role in nephrogenic systemic fibrosis? ( 26336161 )
2015
23
Evidence Suggesting a Role of Iron in a Mouse Model of Nephrogenic Systemic Fibrosis. ( 26305890 )
2015
24
Nephrogenic Systemic Fibrosis on Mammography. ( 25858556 )
2015
25
Diagnosis of Nephrogenic Systemic Fibrosis by means of Elemental Bioimaging and Speciation Analysis. ( 25708271 )
2015
26
Prospective Cohort Study of Nephrogenic Systemic Fibrosis in Patients With Stage 3-5 Chronic Kidney Disease Undergoing MRI With Injected Gadobenate Dimeglumine or Gadoteridol. ( 26295633 )
2015
27
Involvement of the renin-angiotensin system in the development of nephrogenic systemic fibrosis-like lesions in the RenTag mouse model. ( 26138357 )
2015
28
Safety of magnetic resonance contrast media: a review with special focus on nephrogenic systemic fibrosis. ( 25654421 )
2015
29
Compensatory biliary and urinary excretion of gadobenate ion after administration of gadobenate dimeglumine (MultiHance(Ar)) in cases of impaired hepatic or renal function: a mechanism that may aid in the prevention of nephrogenic systemic fibrosis? ( 25651409 )
2015
30
Half-dose gadoxetic acid-enhanced liver magnetic resonance imaging in patients at risk for nephrogenic systemic fibrosis. ( 25591579 )
2015
31
An Updated Study to Determine Association between Gadolinium-Based Contrast Agents and Nephrogenic Systemic Fibrosis. ( 26076348 )
2015
32
Nephrogenic systemic fibrosis following gadolinium administration. ( 26244362 )
2015
33
Application of extracellular gadolinium-based MRI contrast agents and the risk of nephrogenic systemic fibrosis. ( 24477507 )
2014
34
Nephrogenic systemic fibrosis is associated with hypophosphataemia: a case-control study. ( 24706993 )
2014
35
Nephrogenic systemic fibrosis in a gadolinium-naA^ve patient: Successful treatment with oral sirolimus. ( 25330850 )
2014
36
Nephrogenic systemic fibrosis risk and liver disease. ( 24778878 )
2014
37
Incidence of nephrogenic systemic fibrosis in patients undergoing dialysis after contrast-enhanced magnetic resonance imaging with gadolinium-based contrast agents: the prospective fibrose nephrogAcnique systAcmique study. ( 24169070 )
2014
38
Advancing pharmacovigilance through academic-legal collaboration: the case of gadolinium-based contrast agents and nephrogenic systemic fibrosis-a research on adverse drug events and reports (RADAR) report. ( 25230161 )
2014
39
Role of endothelin-1/endothelin receptor signaling in fibrosis and calcification in nephrogenic systemic fibrosis. ( 25048859 )
2014
40
Renal function, nephrogenic systemic fibrosis and other adverse reactions associated with gadolinium-based contrast media. ( 25036056 )
2014
41
Perforating osteoma cutis in the setting of longstanding nephrogenic systemic fibrosis. ( 24962809 )
2014
42
Diagnosing nephrogenic systemic fibrosis in the post-FDA restriction era. ( 24903851 )
2014
43
Risk of nephrogenic systemic fibrosis is low in patients with chronic liver disease exposed to gadolinium-based contrast agents. ( 24811860 )
2014
44
Superior vena cava obstruction associated with nephrogenic systemic fibrosis. ( 24809889 )
2014
45
Current status of nephrogenic systemic fibrosis. ( 24582176 )
2014
46
The role of gadolinium chelates in the mechanism of nephrogenic systemic fibrosis: A critical update. ( 25257840 )
2014
47
Nephrogenic systemic fibrosis in a patient with renal failure demonstrating a "reverse superscan" on bone scintigraphy. ( 23354034 )
2013
48
The treatment of nephrogenic systemic fibrosis with therapeutic plasma exchange. ( 23426620 )
2013
49
Gadolinium and nephrogenic systemic fibrosis: an update. ( 24146299 )
2013
50
Nephrogenic systemic fibrosis and gadolinium-based contrast media: updated ESUR Contrast Medium Safety Committee guidelines. ( 22865271 )
2013

Variations for Nephrogenic Systemic Fibrosis

Expression for Nephrogenic Systemic Fibrosis

Search GEO for disease gene expression data for Nephrogenic Systemic Fibrosis.

Pathways for Nephrogenic Systemic Fibrosis

Pathways related to Nephrogenic Systemic Fibrosis according to GeneCards Suite gene sharing:

(show top 50) (show all 55)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.83 CCL7 CTGF CXCL2 SMAD2 SMAD3 SMAD4
2
Show member pathways
13.67 CCL7 CXCL2 EDN1 EDNRA EPO SMAD3
3
Show member pathways
13.53 CCL7 CTGF EPO PIK3CA SMAD2 SMAD3
4
Show member pathways
13.3 CCL7 CTGF CXCL2 SMAD3 SMAD4 TGFB1
5
Show member pathways
12.83 SMAD2 SMAD3 SMAD4 SMAD7 TGFB1
6 12.83 EDNRA EPO PIK3CA SMAD2 SMAD3 SMAD4
7
Show member pathways
12.76 PIK3CA SMAD2 SMAD3 SMAD4 TGFB1
8
Show member pathways
12.59 EDN1 PIK3CA SMAD2 SMAD3 TGFB1
9
Show member pathways
12.58 CXCL2 PIK3CA SMAD3 SMAD4 TGFB1
10
Show member pathways
12.55 PIK3CA SMAD2 SMAD3 SPP1 TGFB1
11
Show member pathways
12.46 SMAD2 SMAD3 SMAD4 TGFB1
12 12.45 PIK3CA SMAD2 SMAD3 SMAD4 TGFB1
13 12.37 SMAD2 SMAD3 SMAD4 TGFB1
14
Show member pathways
12.35 SMAD2 SMAD3 SMAD4 SMAD7
15
Show member pathways
12.32 SMAD2 SMAD3 SMAD4 SMAD7 TGFB1
16 12.3 DCN PIK3CA SMAD2 TGFB1
17 12.25 CTGF SMAD2 SMAD3 SMAD4 TGFB1
18
Show member pathways
12.24 SMAD2 SMAD3 SMAD4 TGFB1
19 12.19 SMAD2 SMAD3 SMAD4 TGFB1
20 12.18 PIK3CA SMAD2 SMAD3 TGFB1
21
Show member pathways
12.16 PIK3CA SMAD2 SMAD3 SMAD4 TGFB1
22 12.11 PIK3CA SMAD2 SMAD3 SMAD4
23
Show member pathways
12.1 PIK3CA SMAD2 SMAD3 SMAD4 TGFB1
24 12.08 CTGF SMAD2 SMAD3 SMAD4 SPP1
25 12.07 SMAD2 SMAD3 SMAD4 TGFB1
26 12.07 CTGF SMAD2 SMAD3 SMAD4 SMAD7 TGFB1
27 12.06 SMAD2 SMAD3 SMAD4 SMAD7 TGFB1
28 12.01 PIK3CA SMAD3 SMAD4 TGFB1
29 11.94 EDN1 EPO PIK3CA TIMP1
30 11.93 CXCL2 EDN1 PIK3CA
31 11.92 SMAD3 SMAD4 TGFB1
32
Show member pathways
11.9 SMAD2 SMAD3 SMAD4 TGFB1
33
Show member pathways
11.89 SMAD2 SMAD3 SMAD4 TGFB1
34 11.89 SMAD2 SMAD3 SMAD4 SMAD7 TGFB1
35 11.87 DCN SPP1 TGFB1
36
Show member pathways
11.8 SMAD2 SMAD3 SMAD4 TGFB1
37 11.79 SMAD2 SMAD3 SMAD4 SMAD7
38 11.79 EDN1 PIK3CA SMAD2 SMAD3 SMAD4 TGFB1
39 11.73 SMAD2 SMAD3 SMAD4
40 11.71 SMAD2 SMAD3 SMAD4 SMAD7 TGFB1
41 11.7 EDN1 EPO SMAD3 SMAD4
42
Show member pathways
11.68 SMAD2 SMAD3 SMAD4 SMAD7 TGFB1
43 11.66 DCN SMAD2 SMAD3 SMAD4 SMAD7 TGFB1
44
Show member pathways
11.61 PIK3CA SMAD2 SMAD3 SMAD4 TGFB1
45 11.57 SMAD3 SMAD4 TGFB1
46
Show member pathways
11.55 SMAD2 SMAD3 SMAD4
47 11.4 SMAD2 SMAD3 SMAD4 SMAD7 SPP1 TGFB1
48 11.27 SMAD2 SMAD3 SMAD4 TGFB1
49
Show member pathways
11.25 SMAD4 SMAD7 TGFB1
50 11.18 CTGF SMAD2 SMAD3 SMAD4

GO Terms for Nephrogenic Systemic Fibrosis

Cellular components related to Nephrogenic Systemic Fibrosis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 9.91 CCL7 CTGF CXCL2 DCN EDN1 EPO
2 transcription factor complex GO:0005667 9.67 SMAD2 SMAD3 SMAD4 SMAD7
3 platelet alpha granule lumen GO:0031093 9.58 F13A1 TGFB1 TIMP1
4 activin responsive factor complex GO:0032444 9.16 SMAD2 SMAD4
5 heteromeric SMAD protein complex GO:0071144 8.96 SMAD2 SMAD3
6 SMAD protein complex GO:0071141 8.8 SMAD2 SMAD3 SMAD4
7 extracellular region GO:0005576 10.02 CCL7 CTGF CXCL2 DCN EDN1 EPO

Biological processes related to Nephrogenic Systemic Fibrosis according to GeneCards Suite gene sharing:

(show top 50) (show all 54)
# Name GO ID Score Top Affiliating Genes
1 heart development GO:0007507 9.99 EDN1 EDNRA SMAD2 TGFB1
2 positive regulation of ERK1 and ERK2 cascade GO:0070374 9.96 CCL7 CTGF EPO TGFB1
3 positive regulation of cell migration GO:0030335 9.96 CCL7 EDN1 SMAD3 TGFB1
4 response to lipopolysaccharide GO:0032496 9.96 CXCL2 DCN EDN1 EPO
5 aging GO:0007568 9.95 CTGF DCN EPO TGFB1 TIMP1
6 response to organic substance GO:0010033 9.88 SPP1 TGFB1 TIMP1
7 BMP signaling pathway GO:0030509 9.88 SMAD4 SMAD7 TGFB1
8 extracellular matrix disassembly GO:0022617 9.87 DCN SPP1 TIMP1
9 response to glucose GO:0009749 9.86 CTGF SMAD2 TGFB1
10 negative regulation of transforming growth factor beta receptor signaling pathway GO:0030512 9.86 SMAD2 SMAD3 SMAD7 TGFB1
11 regulation of receptor activity GO:0010469 9.86 CCL7 CTGF CXCL2 EDN1 EPO SPP1
12 transforming growth factor beta receptor signaling pathway GO:0007179 9.85 SMAD2 SMAD3 SMAD4 SMAD7 TGFB1
13 in utero embryonic development GO:0001701 9.85 EDN1 EDNRA RCN1 SMAD2 SMAD3 SMAD4
14 SMAD protein signal transduction GO:0060395 9.83 SMAD2 SMAD3 SMAD4 TGFB1
15 wound healing GO:0042060 9.83 DCN SMAD2 SMAD3 TGFB1 TIMP1
16 cellular response to transforming growth factor beta stimulus GO:0071560 9.81 EDN1 SMAD3 SMAD7 TGFB1
17 positive regulation of epithelial to mesenchymal transition GO:0010718 9.8 SMAD2 SMAD3 SMAD4 TGFB1
18 response to hypoxia GO:0001666 9.8 EDN1 EDNRA EPO SMAD3 SMAD4 TGFB1
19 ureteric bud development GO:0001657 9.78 SMAD2 SMAD3 SMAD7 TGFB1
20 gastrulation GO:0007369 9.77 SMAD2 SMAD3 SMAD4
21 developmental growth GO:0048589 9.77 SMAD2 SMAD3 SMAD4
22 activin receptor signaling pathway GO:0032924 9.72 SMAD2 SMAD3
23 negative regulation of cAMP biosynthetic process GO:0030818 9.71 EDN1 EDNRA
24 cell activation GO:0001775 9.71 TGFB1 TIMP1
25 lens fiber cell differentiation GO:0070306 9.71 SMAD3 TGFB1
26 positive regulation of SMAD protein import into nucleus GO:0060391 9.71 SMAD3 SMAD4 TGFB1
27 endoderm development GO:0007492 9.71 SMAD2 SMAD3 SMAD4 TGFB1
28 pathway-restricted SMAD protein phosphorylation GO:0060389 9.7 SMAD7 TGFB1
29 response to dexamethasone GO:0071548 9.7 EDN1 EPO
30 response to cholesterol GO:0070723 9.69 SMAD2 TGFB1
31 primary miRNA processing GO:0031053 9.68 SMAD2 SMAD3
32 embryonic foregut morphogenesis GO:0048617 9.68 SMAD2 SMAD3
33 pericardium development GO:0060039 9.68 SMAD2 SMAD3
34 response to transforming growth factor beta GO:0071559 9.67 EDN1 SMAD4
35 artery smooth muscle contraction GO:0014824 9.67 EDN1 EDNRA
36 response to laminar fluid shear stress GO:0034616 9.67 SMAD7 TGFB1
37 nodal signaling pathway GO:0038092 9.65 SMAD2 SMAD3
38 common-partner SMAD protein phosphorylation GO:0007182 9.64 SMAD2 TGFB1
39 regulation of striated muscle tissue development GO:0016202 9.63 SMAD3 TGFB1
40 response to salt GO:1902074 9.63 EDN1 TGFB1
41 regulation of transforming growth factor beta2 production GO:0032909 9.62 SMAD3 SMAD4
42 positive regulation of extracellular matrix assembly GO:1901203 9.61 SMAD3 TGFB1
43 positive regulation of odontogenesis GO:0042482 9.61 EDN1 TGFB1
44 paraxial mesoderm morphogenesis GO:0048340 9.58 SMAD2 SMAD3
45 evasion or tolerance of host defenses by virus GO:0019049 9.57 SMAD3 TGFB1
46 SMAD protein complex assembly GO:0007183 9.46 SMAD2 SMAD3 SMAD4 TGFB1
47 regulation of transforming growth factor beta receptor signaling pathway GO:0017015 9.35 SMAD2 SMAD3 SMAD4 SMAD7 TGFB1
48 regulation of binding GO:0051098 8.92 SMAD2 SMAD3 SMAD4 TGFB1
49 positive regulation of transcription by RNA polymerase II GO:0045944 10.19 DCN EDN1 SMAD2 SMAD3 SMAD4 SMAD7
50 negative regulation of transcription by RNA polymerase II GO:0000122 10.13 EDN1 EPO SMAD2 SMAD3 SMAD4 SMAD7

Molecular functions related to Nephrogenic Systemic Fibrosis according to GeneCards Suite gene sharing:

(show all 13)
# Name GO ID Score Top Affiliating Genes
1 transforming growth factor beta receptor binding GO:0005160 9.58 SMAD2 SMAD3 TGFB1
2 collagen binding GO:0005518 9.56 DCN SMAD3 SMAD4 SMAD7
3 R-SMAD binding GO:0070412 9.54 SMAD2 SMAD3 SMAD4
4 enhancer binding GO:0035326 9.52 SMAD2 SMAD3
5 co-SMAD binding GO:0070410 9.51 SMAD2 SMAD3
6 protein-glutamine gamma-glutamyltransferase activity GO:0003810 9.49 F13A1 TGM2
7 primary miRNA binding GO:0070878 9.48 SMAD2 SMAD3
8 transforming growth factor beta receptor, pathway-specific cytoplasmic mediator activity GO:0030618 9.46 SMAD2 SMAD3
9 transcription factor activity, protein binding GO:0000988 9.43 SMAD3 SMAD4
10 I-SMAD binding GO:0070411 9.33 SMAD2 SMAD4 SMAD7
11 cytokine activity GO:0005125 9.17 CCL7 CXCL2 EDN1 EPO SPP1 TGFB1
12 type I transforming growth factor beta receptor binding GO:0034713 9.13 SMAD2 SMAD7 TGFB1
13 protein binding GO:0005515 10.28 CCL7 CTGF CXCL2 DCN EDN1 EDNRA

Sources for Nephrogenic Systemic Fibrosis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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