MCID: NPH018
MIFTS: 50

Nephrogenic Systemic Fibrosis

Categories: Rare diseases, Skin diseases, Nephrological diseases

Aliases & Classifications for Nephrogenic Systemic Fibrosis

MalaCards integrated aliases for Nephrogenic Systemic Fibrosis:

Name: Nephrogenic Systemic Fibrosis 50 56 69
Nephrogenic Fibrosing Dermopathy 50 56 69
Nfd 50
Nsf 50

Classifications:

Orphanet: 56  
Rare skin diseases


External Ids:

Orphanet 56 ORPHA137617
UMLS via Orphanet 70 C1619692 C3888044

Summaries for Nephrogenic Systemic Fibrosis

NIH Rare Diseases : 50 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 neuronal intranuclear inclusion disease and tetanus, and has symptoms including exanthema and pruritus. An important gene associated with Nephrogenic Systemic Fibrosis is TGFB1 (Transforming Growth Factor Beta 1), and among its related pathways/superpathways are PEDF Induced Signaling and TGF-Beta Pathway. 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 65)
id Related Disease Score Top Affiliating Genes
1 neuronal intranuclear inclusion disease 11.1
2 tetanus 11.0
3 n syndrome 10.8
4 infant botulism 10.8
5 congenital hypothyroidism, duox2-related 10.6 EDNRA TGFB1
6 urticaria 10.5 SMAD2 SMAD3 TGFB1
7 acromesomelic dysplasia 10.4 SMAD2 SMAD3 SMAD7
8 frozen shoulder 10.4 EDNRA SPP1 TGFB1
9 sarcomatoid renal cell carcinoma 10.4 SMAD2 SMAD3 SMAD4
10 idiopathic steroid-sensitive nephrotic syndrome with diffuse mesangial proliferation 10.3 CTGF SMAD2
11 screw worm infectious disease 10.3 CTGF SPP1 TGFB1
12 keratopathy 10.3 SMAD2 SMAD3 SMAD7 TGFB1
13 kidney disease 10.2
14 bubonic plague 10.1 EDN1 EDNRA TGFB1
15 middle lobe syndrome 10.0 CTGF EDN1 TGFB1
16 cholera 10.0 CTGF TGFB1 TIMP1
17 congenital methemoglobinemia 10.0 SMAD2 SMAD3 TGFB1 TIMP1
18 liver disease 10.0
19 antiphospholipid syndrome 10.0 EDN1 EDNRA
20 exudative vitreoretinopathy 1 10.0 CTGF TGFB1 TIMP1
21 causalgia 10.0 CCL7 CXCL2 SPP1
22 salt and pepper syndrome 10.0 CTGF TGFB1 TIMP1
23 twin-to-twin transfusion syndrome 9.9 DCN SMAD3 SMAD7 TGFB1
24 temporomandibular ankylosis 9.9 CTGF EDN1 TGFB1
25 chronic inflammatory demyelinating polyradiculoneuropathy 9.9 CTGF PIK3CA SMAD2 SMAD3 TGFB1
26 sarcoma 9.9
27 inflammatory breast carcinoma 9.9
28 pulmonary valve insufficiency 9.8 CTGF SMAD2 SMAD3 SMAD7 TGFB1
29 perrault syndrome 9.8 CTGF SMAD2 SMAD3 SMAD7 TGFB1
30 patent foramen ovale 9.8 CTGF SPP1 TGFB1 TIMP1
31 calcinosis 9.8 CTGF EDN1 EPO TGFB1
32 hyperekplexia 2, autosomal recessive 9.8 EDN1 EDNRA
33 craniosynostosis 9.8 EDN1 EDNRA EPO
34 retinitis pigmentosa 9.7
35 retinitis 9.7
36 neuronitis 9.7
37 cocaine dependence 9.7
38 granulomatous orchitis 9.7 EDN1 TGFB1 TIMP1
39 kaposi sarcoma 9.7
40 atherosclerosis 9.7
41 hepatitis 9.7
42 keloids 9.7
43 spinal cord injury 9.7
44 lymphoma 9.7
45 respiratory failure 9.7
46 pure red-cell aplasia 9.7
47 hypothyroidism 9.7
48 panniculitis 9.7
49 myopathy 9.7
50 histiocytoma 9.7

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:


exanthema, pruritus

MGI Mouse Phenotypes related to Nephrogenic Systemic Fibrosis:

44 (show all 21)
id Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 10.5 CXCL2 EDNRA CTGF EDN1 TGFB1 TIMP1
2 cellular MP:0005384 10.4 DCN EDNRA CTGF TGM2 TGFB1 SPP1
3 homeostasis/metabolism MP:0005376 10.4 DCN EDNRA CTGF EDN1 TGM2 TIMP1
4 growth/size/body region MP:0005378 10.36 DCN EDNRA CTGF EDN1 TGM2 TIMP1
5 mortality/aging MP:0010768 10.36 DCN EDNRA CTGF EDN1 TGFB1 TIMP1
6 endocrine/exocrine gland MP:0005379 10.34 DCN EDNRA EDN1 TGFB1 TIMP1 TGM2
7 hematopoietic system MP:0005397 10.31 DCN TGM2 TGFB1 SPP1 EPO F13A1
8 immune system MP:0005387 10.29 DCN SPP1 TGM2 SMAD7 TGFB1 EPO
9 craniofacial MP:0005382 10.28 DCN EDNRA CTGF EDN1 TGFB1 SPP1
10 embryo MP:0005380 10.27 EDNRA CTGF EDN1 TGFB1 SMAD2 PIK3CA
11 integument MP:0010771 10.24 DCN EDNRA CTGF TGFB1 SPP1 SMAD2
12 digestive/alimentary MP:0005381 10.23 DCN EDNRA CTGF EDN1 TGFB1 SMAD3
13 muscle MP:0005369 10.09 EDN1 DCN TGFB1 SPP1 EPO EDNRA
14 neoplasm MP:0002006 10.06 DCN TIMP1 TGFB1 SPP1 TGM2 SMAD2
15 normal MP:0002873 9.97 EDN1 DCN CTGF SMAD7 EPO EDNRA
16 reproductive system MP:0005389 9.96 DCN SPP1 SMAD7 TGFB1 PIK3CA F13A1
17 liver/biliary system MP:0005370 9.95 SPP1 TGFB1 EPO SMAD3 SMAD2 SMAD4
18 renal/urinary system MP:0005367 9.92 EDN1 DCN SPP1 TGM2 TGFB1 EDNRA
19 respiratory system MP:0005388 9.81 DCN EDNRA CTGF TGFB1 SPP1 EPO
20 skeleton MP:0005390 9.73 DCN EDNRA CTGF EDN1 TGFB1 TIMP1
21 vision/eye MP:0005391 9.23 DCN CTGF TGFB1 PIK3CA MAPKAP1 EDNRA

Drugs & Therapeutics for Nephrogenic Systemic Fibrosis

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

(show all 10)
id 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 Investigational 7553-56-2 807
10 cadexomer iodine

Interventional clinical trials:

(show all 27)

id 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 Recruiting NCT02954510 Phase 3
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 Validation of a Questionnaire to Identify Signs and Symptoms of Nephrogenic Systemic Fibrosis Completed NCT00869479
16 Nephrogenic Systemic Fibrosis (NSF): Analysis of Tissue Gadolinium Levels Completed NCT01014754
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 Magnevist Post-marketing Surveillance in Japan Completed NCT01376739 Gadopentetate dimeglumine (Magnevist, BAY86-4882)
22 Adverse Reactions to MR and CT-examinations (Enhanced and Unenhanced) Completed NCT01132339
23 Observational Post-marketing Study on the Safety and Efficacy of Dotarem® (SECURE Study) Completed NCT01523873
24 Pilot Study of the Effect of Laser on Reversing Chronic Radiation Injury Recruiting NCT01910818
25 Evaluation of Gd Retention in Human Bone and Skin After MultiHance or ProHance Administration Compared With Control Recruiting NCT03108378
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:

39
Skin, Kidney, Eye, Testes, Liver, Bone, Heart

Publications for Nephrogenic Systemic Fibrosis

Articles related to Nephrogenic Systemic Fibrosis:

(show top 50) (show all 423)
id Title Authors Year
1
Nephrogenic systemic fibrosis: A 15-year retrospective study at a single tertiary care center. ( 28318680 )
2017
2
Photopheresis Provides Significant Long-Lasting Benefit in Nephrogenic Systemic Fibrosis. ( 28695022 )
2017
3
No Incidence of Nephrogenic Systemic Fibrosis after Gadobenate Dimeglumine Administration in Patients Undergoing Dialysis or Those with Severe Chronic Kidney Disease. ( 28731375 )
2017
4
Erythema nodosum masking nephrogenic systemic fibrosis as initial skin manifestation. ( 28738858 )
2017
5
GBCAs and Risk for Nephrogenic Systemic Fibrosis: A Literature Review. ( 28900045 )
2017
6
Gadolinium-Naive Nephrogenic Systemic Fibrosis of Breast Mimicking Inflammatory Breast Carcinoma. ( 28323787 )
2017
7
Nephrogenic systemic fibrosis. ( 28680623 )
2017
8
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
9
Prophylactic Hemodialysis for Protection Against Gadolinium-Induced Nephrogenic Systemic Fibrosis: A Doll's House. ( 28501073 )
2017
10
Nephrogenic systemic fibrosis: a serious adverse reaction to gadolinium - 1997-2006-2016. Part 1. ( 26802069 )
2016
11
Incidence of Nephrogenic Systemic Fibrosis Using Gadobenate Dimeglumine in 1423 Patients With Renal Insufficiency Compared With Gadodiamide. ( 26885631 )
2016
12
Nephrogenic systemic fibrosis: a serious adverse reaction to gadolinium - 1997-2006-2016. Part 2. ( 26802070 )
2016
13
Interaction of Gd-DTPA with phosphate and phosphite: toward the reaction intermediate in nephrogenic systemic fibrosis. ( 26906409 )
2016
14
Nephrogenic systemic fibrosis: fibrotic plaques and contracture following exposure to gadolinium-based contrast media. ( 27073153 )
2016
15
Nephrogenic Systemic Fibrosis on Mammography. ( 25858556 )
2015
16
Involvement of the renin-angiotensin system in the development of nephrogenic systemic fibrosis-like lesions in the RenTag mouse model. ( 26138357 )
2015
17
Safety of magnetic resonance contrast media: a review with special focus on nephrogenic systemic fibrosis. ( 25654421 )
2015
18
Nephrogenic Systemic Fibrosis Manifesting a Decade After Exposure to Gadolinium. ( 26017458 )
2015
19
Nephrogenic systemic fibrosis following gadolinium administration. ( 26244362 )
2015
20
Half-dose gadoxetic acid-enhanced liver magnetic resonance imaging in patients at risk for nephrogenic systemic fibrosis. ( 25591579 )
2015
21
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
22
An Updated Study to Determine Association between Gadolinium-Based Contrast Agents and Nephrogenic Systemic Fibrosis. ( 26076348 )
2015
23
Evidence Suggesting a Role of Iron in a Mouse Model of Nephrogenic Systemic Fibrosis. ( 26305890 )
2015
24
Do ASARM peptides play a role in nephrogenic systemic fibrosis? ( 26336161 )
2015
25
Gadobenate Dimeglumine Administration and Nephrogenic Systemic Fibrosis: Is There a Real Risk in Patients with Impaired Renal Function? ( 25875973 )
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
Diagnosis of Nephrogenic Systemic Fibrosis by means of Elemental Bioimaging and Speciation Analysis. ( 25708271 )
2015
28
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
29
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
30
Nephrogenic systemic fibrosis in a gadolinium-naA^ve patient: Successful treatment with oral sirolimus. ( 25330850 )
2014
31
Current status of nephrogenic systemic fibrosis. ( 24582176 )
2014
32
Superior vena cava obstruction associated with nephrogenic systemic fibrosis. ( 24809889 )
2014
33
Risk of nephrogenic systemic fibrosis is low in patients with chronic liver disease exposed to gadolinium-based contrast agents. ( 24811860 )
2014
34
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
35
Role of endothelin-1/endothelin receptor signaling in fibrosis and calcification in nephrogenic systemic fibrosis. ( 25048859 )
2014
36
Renal function, nephrogenic systemic fibrosis and other adverse reactions associated with gadolinium-based contrast media. ( 25036056 )
2014
37
Application of extracellular gadolinium-based MRI contrast agents and the risk of nephrogenic systemic fibrosis. ( 24477507 )
2014
38
Nephrogenic systemic fibrosis risk and liver disease. ( 24778878 )
2014
39
Nephrogenic systemic fibrosis is associated with hypophosphataemia: a case-control study. ( 24706993 )
2014
40
The role of gadolinium chelates in the mechanism of nephrogenic systemic fibrosis: A critical update. ( 25257840 )
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
A difficult nasogastric tube in a patient with nephrogenic systemic fibrosis. ( 23857072 )
2013
44
Antifibrotic effect after low-dose imatinib mesylate treatment in patients with nephrogenic systemic fibrosis: an open-label non-randomized, uncontrolled clinical trial. ( 22188390 )
2013
45
The treatment of nephrogenic systemic fibrosis with therapeutic plasma exchange. ( 23426620 )
2013
46
Evaluation of imatinib mesylate as a possible treatment for nephrogenic systemic fibrosis in a rat model. ( 22898683 )
2013
47
The debate between gadolinium versus erythropoietin in a renal transplant patient with nephrogenic systemic fibrosis. ( 22573525 )
2013
48
Gadolinium contrast agent-induced CD163+ ferroportin+ osteogenic cells in nephrogenic systemic fibrosis. ( 23867799 )
2013
49
Pediatric nephrogenic systemic fibrosis is rarely reported: a RADAR report. ( 24057195 )
2013
50
Nephrogenic systemic fibrosis in a patient with renal failure demonstrating a "reverse superscan" on bone scintigraphy. ( 23354034 )
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 all 46)
id Super pathways Score Top Affiliating Genes
1
Show member pathways
13.65 CCL7 CXCL2 EDN1 EDNRA EPO SMAD3
2
Show member pathways
13.51 CCL7 CTGF EPO PIK3CA SMAD2 SMAD3
3
Show member pathways
13.28 CCL7 CTGF CXCL2 SMAD3 SMAD4 TGFB1
4
Show member pathways
12.85 PIK3CA SMAD2 SMAD3 SPP1 TGFB1
5
Show member pathways
12.81 SMAD2 SMAD3 SMAD4 SMAD7 TGFB1
6 12.68 EDNRA PIK3CA SMAD2 SMAD3 SMAD4 TGFB1
7
Show member pathways
12.44 SMAD2 SMAD3 SMAD4 TGFB1
8 12.44 PIK3CA SMAD2 SMAD3 SMAD4 TGFB1
9 12.35 SMAD2 SMAD3 SMAD4 TGFB1
10
Show member pathways
12.33 SMAD2 SMAD3 SMAD4 SMAD7
11
Show member pathways
12.29 SMAD2 SMAD3 SMAD4 SMAD7 TGFB1
12 12.28 DCN PIK3CA SMAD2 TGFB1
13 12.23 SMAD2 SMAD3 SMAD4 TGFB1
14
Show member pathways
12.21 SMAD2 SMAD3 SMAD4 TGFB1
15 12.17 SMAD2 SMAD3 SMAD4 TGFB1
16
Show member pathways
12.1 PIK3CA SMAD2 SMAD3 SMAD4 TGFB1
17 12.09 PIK3CA SMAD2 SMAD3 SMAD4
18 12.09 CTGF SMAD2 SMAD3 SMAD4 SPP1
19 12.07 CTGF SMAD2 SMAD3 SMAD4 SMAD7 TGFB1
20 12.06 SMAD2 SMAD3 SMAD4 SMAD7 TGFB1
21 12.05 SMAD2 SMAD3 SMAD4 TGFB1
22 12 PIK3CA SMAD3 SMAD4 TGFB1
23 11.92 EDN1 EPO PIK3CA TIMP1
24
Show member pathways
11.92 SMAD2 SMAD3 SMAD4 TGFB1
25 11.91 SMAD2 SMAD3 SMAD4 SMAD7 TGFB1
26 11.9 SMAD3 SMAD4 TGFB1
27
Show member pathways
11.89 SMAD2 SMAD3 SMAD4 TGFB1
28 11.85 DCN SPP1 TGFB1
29 11.79 EDN1 PIK3CA SMAD2 SMAD3 SMAD4 TGFB1
30 11.77 SMAD2 SMAD3 SMAD4 SMAD7
31 11.72 SMAD2 SMAD3 SMAD4
32 11.71 SMAD2 SMAD3 SMAD4 SMAD7 TGFB1
33 11.68 EDN1 EPO SMAD3 SMAD4
34
Show member pathways
11.68 SMAD2 SMAD3 SMAD4 SMAD7 TGFB1
35 11.66 DCN SMAD2 SMAD3 SMAD4 SMAD7 TGFB1
36
Show member pathways
11.61 PIK3CA SMAD2 SMAD3 SMAD4 TGFB1
37 11.54 SMAD3 SMAD4 TGFB1
38
Show member pathways
11.53 SMAD2 SMAD3 SMAD4
39 11.4 SMAD2 SMAD3 SMAD4 SMAD7 SPP1 TGFB1
40 11.27 SMAD2 SMAD3 SMAD4 TGFB1
41
Show member pathways
11.24 SMAD4 SMAD7 TGFB1
42 11.18 CTGF SMAD2 SMAD3 SMAD4
43 11.14 CTGF SMAD2 SMAD3 SMAD4 SMAD7 TGFB1
44 11.05 SMAD2 SMAD3 SMAD4
45 10.97 CTGF CXCL2 EDN1 SMAD7 SPP1 TGFB1
46 10.92 SMAD2 SMAD3 SMAD4 TGFB1

GO Terms for Nephrogenic Systemic Fibrosis

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

id 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 SMAD2-SMAD3 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 52)
id Name GO ID Score Top Affiliating Genes
1 heart development GO:0007507 9.98 EDN1 EDNRA SMAD2 TGFB1
2 positive regulation of cell migration GO:0030335 9.95 CCL7 EDN1 SMAD3 TGFB1
3 positive regulation of ERK1 and ERK2 cascade GO:0070374 9.95 CCL7 CTGF EPO TGFB1
4 response to lipopolysaccharide GO:0032496 9.94 CXCL2 DCN EDN1 EPO
5 inflammatory response GO:0006954 9.93 CCL7 CXCL2 SPP1 TGFB1
6 aging GO:0007568 9.93 CTGF DCN EPO TGFB1 TIMP1
7 response to organic substance GO:0010033 9.88 SPP1 TGFB1 TIMP1
8 BMP signaling pathway GO:0030509 9.87 SMAD4 SMAD7 TGFB1
9 extracellular matrix disassembly GO:0022617 9.86 DCN SPP1 TIMP1
10 response to glucose GO:0009749 9.85 CTGF SMAD2 TGFB1
11 in utero embryonic development GO:0001701 9.85 EDN1 EDNRA RCN1 SMAD2 SMAD3 SMAD4
12 negative regulation of transforming growth factor beta receptor signaling pathway GO:0030512 9.83 SMAD2 SMAD3 SMAD7 TGFB1
13 SMAD protein signal transduction GO:0060395 9.81 SMAD2 SMAD3 SMAD4 TGFB1
14 cellular response to transforming growth factor beta stimulus GO:0071560 9.8 EDN1 SMAD3 SMAD7 TGFB1
15 transforming growth factor beta receptor signaling pathway GO:0007179 9.8 SMAD2 SMAD3 SMAD4 SMAD7 TGFB1
16 positive regulation of epithelial to mesenchymal transition GO:0010718 9.78 SMAD2 SMAD3 SMAD4 TGFB1
17 gastrulation GO:0007369 9.76 SMAD2 SMAD3 SMAD4
18 ureteric bud development GO:0001657 9.76 SMAD2 SMAD3 SMAD7 TGFB1
19 developmental growth GO:0048589 9.75 SMAD2 SMAD3 SMAD4
20 wound healing GO:0042060 9.73 DCN SMAD3 TGFB1 TIMP1
21 response to hypoxia GO:0001666 9.73 EDN1 EDNRA EPO SMAD3 SMAD4 TGFB1
22 activin receptor signaling pathway GO:0032924 9.71 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 9.7 SMAD3 TGFB1
26 pathway-restricted SMAD protein phosphorylation GO:0060389 9.7 SMAD7 TGFB1
27 positive regulation of SMAD protein import into nucleus GO:0060391 9.69 SMAD4 TGFB1
28 response to dexamethasone GO:0071548 9.69 EDN1 EPO
29 primary miRNA processing GO:0031053 9.68 SMAD2 SMAD3
30 pericardium development GO:0060039 9.67 SMAD2 SMAD3
31 response to cholesterol GO:0070723 9.67 SMAD2 TGFB1
32 embryonic foregut morphogenesis GO:0048617 9.67 SMAD2 SMAD3
33 response to transforming growth factor beta GO:0071559 9.66 EDN1 SMAD4
34 artery smooth muscle contraction GO:0014824 9.66 EDN1 EDNRA
35 response to laminar fluid shear stress GO:0034616 9.65 SMAD7 TGFB1
36 nodal signaling pathway GO:0038092 9.64 SMAD2 SMAD3
37 common-partner SMAD protein phosphorylation GO:0007182 9.62 SMAD2 TGFB1
38 regulation of striated muscle tissue development GO:0016202 9.62 SMAD3 TGFB1
39 endoderm development GO:0007492 9.62 SMAD2 SMAD3 SMAD4 TGFB1
40 positive regulation of extracellular matrix assembly GO:1901203 9.61 SMAD3 TGFB1
41 regulation of transforming growth factor beta2 production GO:0032909 9.6 SMAD3 SMAD4
42 positive regulation of odontogenesis GO:0042482 9.59 EDN1 TGFB1
43 paraxial mesoderm morphogenesis GO:0048340 9.56 SMAD2 SMAD3
44 evasion or tolerance of host defenses by virus GO:0019049 9.55 SMAD3 TGFB1
45 SMAD protein complex assembly GO:0007183 9.46 SMAD2 SMAD3 SMAD4 TGFB1
46 regulation of transforming growth factor beta receptor signaling pathway GO:0017015 9.35 SMAD2 SMAD3 SMAD4 SMAD7 TGFB1
47 regulation of binding GO:0051098 8.92 SMAD2 SMAD3 SMAD4 TGFB1
48 positive regulation of transcription from RNA polymerase II promoter GO:0045944 10.18 DCN EDN1 SMAD2 SMAD3 SMAD4 SMAD7
49 negative regulation of transcription from RNA polymerase II promoter GO:0000122 10.11 EDN1 EPO SMAD2 SMAD3 SMAD4 SMAD7
50 positive regulation of cell proliferation GO:0008284 10.09 CTGF EDN1 EPO TGFB1 TIMP1

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

(show all 13)
id 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 type I transforming growth factor beta receptor binding GO:0034713 9.13 SMAD2 SMAD7 TGFB1
12 cytokine activity GO:0005125 9.1 CCL7 CXCL2 EDN1 SPP1 TGFB1 TIMP1
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
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 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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