NFD
MCID: NPH018
MIFTS: 48

Nephrogenic Systemic Fibrosis (NFD)

Categories: Nephrological diseases, Rare diseases, Skin diseases

Aliases & Classifications for Nephrogenic Systemic Fibrosis

MalaCards integrated aliases for Nephrogenic Systemic Fibrosis:

Name: Nephrogenic Systemic Fibrosis 20 58 70
Nephrogenic Fibrosing Dermopathy 20 58 17 70
Nfd 20
Nsf 20

Classifications:

Orphanet: 58  
Rare skin diseases


External Ids:

UMLS via Orphanet 71 C1619692 C3888044
Orphanet 58 ORPHA137617
UMLS 70 C1619692 C3888044

Summaries for Nephrogenic Systemic Fibrosis

GARD : 20 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.

MalaCards based summary : Nephrogenic Systemic Fibrosis, also known as nephrogenic fibrosing dermopathy, is related to scleroderma, familial progressive and chronic kidney disease, and has symptoms including pruritus and exanthema. An important gene associated with Nephrogenic Systemic Fibrosis is DCN (Decorin), and among its related pathways/superpathways are ERK Signaling and PEDF Induced Signaling. The drugs Pharmaceutical Solutions and Imatinib Mesylate have been mentioned in the context of this disorder. Affiliated tissues include kidney, heart and skin, and related phenotypes are cardiovascular system and cellular

Wikipedia : 73 Nephrogenic systemic fibrosis is a rare syndrome that involves fibrosis of skin, joints, eyes, and... 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 184)
# Related Disease Score Top Affiliating Genes
1 scleroderma, familial progressive 31.2 EDN1 CCN2
2 chronic kidney disease 30.3 TGFB1 SPP1 EPO EDN1 CCN2
3 kidney disease 30.0 TGFB1 SPP1 EPO EDNRA EDN1 CCN2
4 glomerulonephritis 29.5 TGFB1 SPP1 SMAD2 CCN2
5 pulmonary fibrosis 29.4 TIMP1 TGFB1 SMAD7 SMAD3 EDN1 CCN2
6 localized scleroderma 29.1 TGFB1 SMAD7 SMAD4 SMAD3 DCN CCN2
7 tetanus 11.2
8 schizophrenia 11.1
9 fibrosis of extraocular muscles, congenital, 1 11.0
10 neuronal intranuclear inclusion disease 10.9
11 early infantile epileptic encephalopathy 10.9
12 type 1 diabetes mellitus 13 10.9
13 leukodystrophy, hypomyelinating, 6 10.9
14 tungiasis 10.9
15 syndromic x-linked intellectual disability snyder type 10.9
16 end stage renal disease 10.7
17 acute kidney failure 10.6
18 systemic scleroderma 10.6
19 scleromyxedema 10.6
20 dowling-degos disease 1 10.4
21 metabolic acidosis 10.3
22 systemic lupus erythematosus 10.3
23 pseudoxanthoma elasticum 10.3
24 acute kidney tubular necrosis 10.3
25 lupus erythematosus 10.3
26 polykaryocytosis inducer 10.3
27 hyperphosphatemia 10.3
28 hepatorenal syndrome 10.3
29 scleredema adultorum 10.3
30 skin disease 10.3
31 stachybotrys chartarum 10.3 CXCL2 CCL7
32 malignant renovascular hypertension 10.2 EDNRA EDN1
33 urethral stricture 10.2 TGFB1 CCN2
34 pauci-immune glomerulonephritis 10.2 SMAD2 CCN2
35 diffuse scleroderma 10.2 TGFB1 EDN1 CCN2
36 kaposi sarcoma 10.2
37 erythema nodosum 10.2
38 sarcoma 10.2
39 hyperparathyroidism 10.2
40 spindle cell sarcoma 10.2
41 calciphylaxis 10.2
42 femoral cancer 10.2 EDNRA EDN1
43 hypertensive encephalopathy 10.2 EPO EDN1
44 cholecystolithiasis 10.2 TGFB1 CCN2
45 idiopathic interstitial pneumonia 10.2 TGFB1 EDN1 CCN2
46 perinephritis 10.2 EDNRA EDN1
47 transient refractive change 10.2 EDNRA EDN1
48 acquired color blindness 10.2 EPO EDNRA EDN1
49 limited scleroderma 10.2 EDNRA EDN1 CCN2
50 dermatomyositis 10.1

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:

46 (show all 22)
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 10.53 CCN2 CXCL2 EDN1 EDNRA EPO F13A1
2 cellular MP:0005384 10.4 CCN2 DCN EDNRA EPO MAPKAP1 PIK3CA
3 homeostasis/metabolism MP:0005376 10.4 CCN2 DCN EDN1 EDNRA EPO F13A1
4 growth/size/body region MP:0005378 10.39 CCN2 DCN EDN1 EDNRA MAPKAP1 PIK3CA
5 behavior/neurological MP:0005386 10.38 DCN EDNRA F13A1 PIK3CA RCN1 SMAD2
6 endocrine/exocrine gland MP:0005379 10.37 CCN2 DCN EDN1 EDNRA F13A1 PIK3CA
7 mortality/aging MP:0010768 10.36 CCN2 DCN EDN1 EDNRA EPO F13A1
8 hematopoietic system MP:0005397 10.35 CXCL2 DCN EDNRA EPO F13A1 SMAD2
9 immune system MP:0005387 10.32 CXCL2 DCN EDNRA EPO PIK3CA SMAD2
10 embryo MP:0005380 10.31 CCN2 EDN1 EDNRA EPO MAPKAP1 PIK3CA
11 craniofacial MP:0005382 10.28 CCN2 DCN EDN1 EDNRA SMAD2 SMAD3
12 integument MP:0010771 10.27 CCN2 DCN EDNRA EPO F13A1 PIK3CA
13 digestive/alimentary MP:0005381 10.23 CCN2 DCN EDN1 EDNRA SMAD2 SMAD3
14 muscle MP:0005369 10.09 DCN EDN1 EDNRA EPO PIK3CA SMAD4
15 neoplasm MP:0002006 10.06 DCN PIK3CA SMAD2 SMAD3 SMAD4 SPP1
16 liver/biliary system MP:0005370 10.02 CCN2 EPO SMAD2 SMAD3 SMAD4 SPP1
17 normal MP:0002873 9.97 CCN2 DCN EDN1 EDNRA EPO SMAD2
18 reproductive system MP:0005389 9.96 DCN F13A1 PIK3CA SMAD2 SMAD3 SMAD4
19 renal/urinary system MP:0005367 9.92 DCN EDN1 EDNRA SMAD3 SMAD4 SPP1
20 respiratory system MP:0005388 9.81 CCN2 DCN EDNRA EPO F13A1 SMAD2
21 skeleton MP:0005390 9.8 CCN2 CXCL2 DCN EDN1 EDNRA EPO
22 vision/eye MP:0005391 9.36 CCN2 CXCL2 DCN EDNRA MAPKAP1 PIK3CA

Drugs & Therapeutics for Nephrogenic Systemic Fibrosis

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


# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Pharmaceutical Solutions Phase 4
2 Imatinib Mesylate Phase 3 220127-57-1 123596
3 Protein Kinase Inhibitors Phase 3

Interventional clinical trials:

(show all 15)
# Name Status NCT ID Phase Drugs
1 Prospective Non-randomized (Pharmacoepidemiologic) Cohort Study (Open-label, Multicenter) to Assess the Magnitude of Potential Risk With the Administration of Primovist/Eovist in Patients With Moderate to Severe Renal Impairment for the Development of Nephrogenic Systemic Fibrosis (NSF) Based on Diagnostically Specific Clinical and Histopathologic Information. Completed NCT00908596 Phase 4 Gadoxetic acid disodium (Primovist, BAY86-4873)
2 Prospective Non-randomized (Pharmacoepidemiologic) Cohort Study (Open-label, Multicenter) to Assess the Magnitude of Potential Risk With the Administration of Gadovist in Patients With Moderate to Severe Renal Impairment for the Development of Nephrogenic Systemic Fibrosis (NSF) Based on Diagnostically Specific Clinical and Histopathologic Information Completed NCT00828737 Phase 4 Gadobutrol (Gadovist, BAY86-4875)
3 Safety Evaluation of Linear and Macrocyclic Gadolinium-Based Contrast Agents for Patients With Mild to Moderate Renal Insufficiency Undergoing Enhanced Magnetic Resonance Imaging (Interventional Prospective Study) Clinical Trial Protocol Recruiting NCT04776187 Phase 4 Gadodiamide;Gadoteric Acid Meglumine Salt
4 Use of Plasmapheresis for Nephrogenic Fibrosing Dermopathy (NFD)/Nephrogenic Systemic Fibrosis (NSF) and Understanding Its Etiology Terminated NCT01078987 Phase 4
5 The Prevalence and Severity of Nephrogenic Systemic Fibrosis in Patients Whom Underwent Imaging With Gadollinium(MRI/MRA) in University Hospital of Tehran Heart Center in 2003-2008 Unknown status NCT01359345 Phase 2, Phase 3 gadollinum
6 An Open Label Clinical Trial of Imatinib Mesylate(Glivec)in Patients With Moderate to Severe Nephrogenic Systemic Fibrosis Completed NCT00981942 Phase 3 Imatinib mesylate (Glivec)
7 An Open Label Phase 2 Pilot Study to Determine the Safety, Efficacy and Tolerability of Gleevec (Imatinib Mesylate) in the Treatment of Nephrogenic Systemic Fibrosis Completed NCT00677092 Phase 2 Imatinib mesylate
8 Nephrogenic Systemic Fibrosis and Gadolinium-A Medical Record Review With Analysis of Existing Skin and Other Tissue Specimens to Assess Potential Causative or Associated Factors Completed NCT01014754
9 Validation of a Questionnaire as a Screening Tool to Identify Signs and Symptoms of Nephrogenic Systemic Fibrosis Completed NCT00869479
10 A Phase 4, Open-Label, Prospective Evaluation of the Incidence of Nephrogenic Systemic Fibrosis (NSF) in Patients With Moderate to Severe Kidney Disease Undergoing Magnetic Resonance Imaging (MRI) With Ablavar (Gadofosveset Trisodium) in Routine Clinical Practice Completed NCT01135316 Ablavar
11 A Prospective Study Evaluating the Incidence of Nephrogenic Systemic Fibrosis in Patients With Stages 4 to 5 Chronic Kidney Disease Without Exposure to Gadolinium Based Contrast Agents Within the Past 10 Years Completed NCT00773409
12 Prospective Non-randomized Observational (Pharmacoepidemiologic) Cohort Study (Open-label, Multicenter) to Assess the Magnitude of Potential Risk With the Administration of Magnevist® Injection in Patients With Moderate Renal Impairment for the Development of Nephrogenic Systemic Fibrosis (NSF) Based on Diagnostically Specific Clinical and Histopathologic Information. Completed NCT00744939 Gadopentetate dimeglumine (Magnevist, BAY86-4882)
13 A Prospective Cohort Study Evaluating the Incidence of Nephrogenic Systemic Fibrosis in Patients With Stages 3 to 5 Chronic Kidney Disease Undergoing MRI With the Injection of MultiHance Completed NCT00600951
14 A Prospective Cohort Study Evaluating the Incidence of Nephrogenic Systemic Fibrosis in Patients With Stages 3 to 5 Chronic Kidney Disease Undergoing MRI With the Injection of ProHance Completed NCT00600834
15 A Prospective Study for the Detection of Nephrogenic Systemic Fibrosis in Patients With Renal Impairment Undergoing Gadoversetamide-enhanced Magnetic Resonance Imaging 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:

40
Kidney, Heart, Skin, Liver, Bone Marrow, Skeletal Muscle, Breast

Publications for Nephrogenic Systemic Fibrosis

Articles related to Nephrogenic Systemic Fibrosis:

(show top 50) (show all 990)
# Title Authors PMID Year
1
Risks and Options With Gadolinium-Based Contrast Agents in Patients With CKD: A Review. 61
32861792 2021
2
Nephrogenic Systemic Fibrosis as a Complication after Gadolinium-Containing Contrast Agents: A Rapid Review. 61
33804005 2021
3
The Use of Contrast Agents in Interventional Pain Procedures: A Multispecialty and Multisociety Practice Advisory on Nephrogenic Systemic Fibrosis, Gadolinium Deposition in the Brain, Encephalopathy After Unintentional Intrathecal Gadolinium Injection, and Hypersensitivity Reactions. 61
33755647 2021
4
Risk of nephrogenic systemic fibrosis in patients with impaired renal function undergoing fixed-dose gadoxetic acid-enhanced magnetic resonance imaging. 61
33742216 2021
5
Dynamic Contrast Enhanced-MR CEST Urography: An Emerging Tool in the Diagnosis and Management of Upper Urinary Tract Obstruction. 61
33801533 2021
6
Mn2+ Complexes with Pyclen-Based Derivatives as Contrast Agents for Magnetic Resonance Imaging: Synthesis and Relaxometry Characterization. 61
33625836 2021
7
Interference of gadolinium dechelated from MR contrast agents by calcium signaling in neuronal cells of GnRH. 61
32740939 2021
8
Acquired reactive perforating collagenosis in a patient with chronic renal failure and nephrogenic systemic fibrosis. 61
32812216 2021
9
Nephrogenic Systemic Fibrosis. 61
32709396 2021
10
Noncontrast Magnetic Resonance Angiography in the Era of Nephrogenic Systemic Fibrosis and Gadolinium Deposition. 61
32976265 2021
11
Diagnostic Imaging: Appropriate and Safe Use. 61
33382559 2021
12
Use of Intravenous Gadolinium-based Contrast Media in Patients with Kidney Disease: Consensus Statements from the American College of Radiology and the National Kidney Foundation. 61
33170103 2021
13
Use of Intravenous Gadolinium-Based Contrast Media in Patients With Kidney Disease: Consensus Statements from the American College of Radiology and the National Kidney Foundation. 61
33604544 2021
14
Folic acid-appended galactoxyloglucan-capped iron oxide nanoparticles as a biocompatible nanotheranostic agent for tumor-targeted delivery of doxorubicin. 61
33278441 2021
15
Benefits and Detriments of Gadolinium from Medical Advances to Health and Ecological Risks. 61
33297578 2020
16
No Cases of Nephrogenic Systemic Fibrosis after Administration of Gadoxetic Acid. 61
32990511 2020
17
Risk of Nephrogenic Systemic Fibrosis from Gadoxetic Acid in Patients with Severe Kidney Disease. 61
32996871 2020
18
Gadolinium-based contrast agents: What we learned from acute adverse events, nephrogenic systemic fibrosis and brain retention. 61
33348385 2020
19
Chemical exposure-induced systemic fibrosing disorders: Novel insights into systemic sclerosis etiology and pathogenesis. 61
33059296 2020
20
Adverse Events to the Gadolinium-based Contrast Agent Gadoxetic Acid: Systematic Review and Meta-Analysis. 61
32452732 2020
21
Consensus Guidelines of the French Society of Neuroradiology (SFNR) on the use of Gadolinium-Based Contrast agents (GBCAs) and related MRI protocols in Neuroradiology. 61
32565280 2020
22
Risk of Nephrogenic Systemic Fibrosis in Stage 4 and 5 Chronic Kidney Disease Following Group II Gadolinium-based Contrast Agent Administration: Subanalysis by Chronic Kidney Disease Stage. 61
32808890 2020
23
MR Imaging Safety Considerations of Gadolinium-Based Contrast Agents: Gadolinium Retention and Nephrogenic Systemic Fibrosis. 61
33040991 2020
24
The Risk of Nephrogenic Systemic Fibrosis in Group II and III Gadolinium-Based Contrast Agents. 61
32063014 2020
25
Contrast media in patients with kidney disease: An update. 61
33139262 2020
26
Identification and characterization of Gd-binding proteins in NIH-3T3 cells. 61
32887171 2020
27
A nephrotoxicity-free, iron-based contrast agent for magnetic resonance imaging of tumors. 61
32736259 2020
28
Current and Future MR Contrast Agents: Seeking a Better Chemical Stability and Relaxivity for Optimal Safety and Efficacy. 61
32776767 2020
29
Late Onset Nephrogenic Systemic Fibrosis in a Patient with Stage 3 Chronic Kidney Disease: a Case Report. 61
32893521 2020
30
N-acetylcysteine-PLGA nano-conjugate: effects on cellular toxicity and uptake of gadopentate dimeglumine. 61
32755956 2020
31
Practical recommendations for the safe use of gadolinium in magnetic resonance imaging: a Delphi expert panel study. 61
32904664 2020
32
Incidence of nephrogenic systemic fibrosis after administration of gadoteric acid in patients on renal replacement treatment. 61
32112811 2020
33
Risk for Nephrogenic Systemic Fibrosis After Exposure to Newer Gadolinium Agents: A Systematic Review. 61
32568573 2020
34
Strategic reconstruction of macrophage-derived extracellular vesicles as a magnetic resonance imaging contrast agent. 61
32300771 2020
35
Risks and Benefits of Gadolinium-Based Contrast-Enhanced MRI. 61
32446430 2020
36
Multimodal indirect imaging signs of pulmonary embolism. 61
31944831 2020
37
Gadolinium-based contrast agents - what is the evidence for 'gadolinium deposition disease' and the use of chelation therapy? 61
31663374 2020
38
Nephrogenic Systemic Fibrosis Risk Assessment and Skin Biopsy Quantification in Patients with Renal Disease following Gadobenate Contrast Administration. 61
32115422 2020
39
Sclerodermalike syndromes: Great imitators. 61
32513403 2020
40
Risk of Nephrogenic Systemic Fibrosis in Patients With Stage 4 or 5 Chronic Kidney Disease Receiving a Group II Gadolinium-Based Contrast Agent: A Systematic Review and Meta-analysis. 61
31816007 2020
41
Observational study on the incidence of nephrogenic systemic fibrosis in patients with renal impairment following gadoterate meglumine administration: the NSsaFe study. 61
31287213 2020
42
Noncontrast Magnetic Resonance Angiography Clinical Application in Pre-Liver Transplant Recipients With Impaired Renal Function. 61
31715655 2020
43
Gadolinium Retention and Breast MRI Screening: More Harm Than Good? 61
31799869 2020
44
Radiocontrast Toxicity. 61
32147002 2020
45
Integration of gadolinium in nanostructure for contrast enhanced-magnetic resonance imaging. 61
31486295 2020
46
Feasibility of using half-dose Gd-BOPTA for delayed gadolinium-enhanced MRI of cartilage (dGEMRIC) at the knee, compared with standard-dose Gd-DTPA. 61
31141254 2020
47
Gadolinium Deposition and Nephrogenic Systemic Fibrosis: A Radiologist's Primer. 61
31809230 2020
48
The first case of coexistence of calciphylaxis and nephrogenic systemic fibrosis with different localizations in a hemodialysis patient. 61
31182286 2020
49
Is it time to relax nephrogenic systemic fibrosis guidelines and safely offer magnetic resonance imaging to more patients? 61
31867244 2019
50
The extra miles on preventing nephrogenic systemic fibrosis. 61
31867242 2019

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 57)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.82 TIMP1 TGFB1 SPP1 SMAD4 SMAD3 SMAD2
2
Show member pathways
13.68 TIMP1 TGFB1 SMAD4 SMAD3 EPO EDNRA
3
Show member pathways
13.51 TIMP1 TGFB1 SMAD7 SMAD4 SMAD3 SMAD2
4
Show member pathways
13.3 TIMP1 TGFB1 SMAD4 SMAD3 CXCL2 CCN2
5
Show member pathways
13.2 TGFB1 SPP1 SMAD4 SMAD3 SMAD2 PIK3CA
6
Show member pathways
12.82 TGFB1 SMAD7 SMAD4 SMAD3 SMAD2
7 12.8 TGFB1 SMAD4 SMAD3 SMAD2 PIK3CA EPO
8
Show member pathways
12.79 TGFB1 SMAD4 SMAD3 SMAD2 PIK3CA
9
Show member pathways
12.76 TGFB1 SMAD4 SMAD3 SMAD2 PIK3CA
10
Show member pathways
12.5 TGFB1 SMAD4 SMAD3 SMAD2
11
Show member pathways
12.46 TGFB1 SMAD4 SMAD3 SMAD2
12 12.36 TGFB1 SMAD4 SMAD3 SMAD2
13 12.36 TGFB1 SMAD4 SMAD3 SMAD2 PIK3CA
14
Show member pathways
12.35 SMAD7 SMAD4 SMAD3 SMAD2
15 12.3 TGFB1 SMAD2 PIK3CA DCN
16
Show member pathways
12.3 TGFB1 SMAD7 SMAD4 SMAD3 SMAD2
17 12.25 TGFB1 SMAD4 SMAD3 SMAD2 CCN2
18 12.19 TGFB1 SMAD4 SMAD3 SMAD2
19 12.16 TGFB1 SMAD3 SMAD2 PIK3CA
20 12.12 SMAD4 SMAD3 SMAD2 PIK3CA
21
Show member pathways
12.1 TGFB1 SMAD4 SMAD3 PIK3CA
22 12.1 SPP1 SMAD4 SMAD3 SMAD2 CCN2
23 12.08 TGFB1 SMAD7 SMAD4 SMAD3 SMAD2
24 12.08 TGFB1 SMAD7 SMAD4 SMAD3 SMAD2 CCN2
25 12.01 TGFB1 SMAD4 SMAD3 PIK3CA
26 11.98 TIMP1 PIK3CA EPO EDN1
27
Show member pathways
11.98 TGFB1 SMAD4 SMAD3 SMAD2
28 11.95 PIK3CA EDN1 CXCL2
29 11.92 TGFB1 SMAD4 SMAD3
30 11.92 TGFB1 SMAD7 SMAD4 SMAD3 SMAD2
31 11.9 TGFB1 PIK3CA CXCL2
32
Show member pathways
11.9 TGFB1 SMAD4 SMAD3 SMAD2
33
Show member pathways
11.89 TGFB1 SMAD4 SMAD3 SMAD2
34 11.87 TGFB1 SPP1 DCN
35
Show member pathways
11.8 TGFB1 SMAD4 SMAD3 SMAD2
36 11.8 TGFB1 SMAD4 SMAD3 SMAD2 PIK3CA EDN1
37 11.79 SMAD7 SMAD4 SMAD3 SMAD2
38 11.75 TGFB1 SMAD7 SMAD4 SMAD3 SMAD2
39 11.72 TGFB1 SMAD4 SMAD3 SMAD2
40 11.71 TGFB1 SMAD7 SMAD4 SMAD3 SMAD2 DCN
41 11.7 SMAD4 SMAD3 EPO EDN1
42 11.7 SMAD7 SMAD4 SMAD3 SMAD2 PIK3CA
43
Show member pathways
11.68 TGFB1 SMAD7 SMAD4 SMAD3 SMAD2
44
Show member pathways
11.64 TGFB1 SMAD4 SMAD3 SMAD2 PIK3CA
45 11.56 TGFB1 SMAD4 SMAD3
46
Show member pathways
11.55 SMAD4 SMAD3 SMAD2
47 11.4 TGFB1 SPP1 SMAD7 SMAD4 SMAD3 SMAD2
48 11.3 TGFB1 SMAD4 SMAD3 SMAD2
49
Show member pathways
11.25 TGFB1 SMAD7 SMAD4
50
Show member pathways
11.21 SMAD4 SMAD3 PIK3CA

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 9.97 TIMP1 TGFB1 SPP1 EPO EDN1 DCN
2 extracellular region GO:0005576 9.96 TIMP1 TGFB1 SPP1 F13A1 EPO EDN1
3 collagen-containing extracellular matrix GO:0062023 9.77 TGM2 TGFB1 F13A1 DCN CCN2
4 extracellular matrix GO:0031012 9.76 TIMP1 TGFB1 DCN CCN2
5 transcription factor complex GO:0005667 9.73 SMAD7 SMAD4 SMAD3 SMAD2
6 platelet alpha granule lumen GO:0031093 9.58 TIMP1 TGFB1 F13A1
7 activin responsive factor complex GO:0032444 9.16 SMAD4 SMAD2
8 SMAD protein complex GO:0071141 9.13 SMAD4 SMAD3 SMAD2
9 heteromeric SMAD protein complex GO:0071144 8.92 SMAD7 SMAD4 SMAD3 SMAD2

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

(show top 50) (show all 61)
# Name GO ID Score Top Affiliating Genes
1 cell differentiation GO:0030154 10.2 SPP1 SMAD7 SMAD4 SMAD3 SMAD2 CCN2
2 positive regulation of transcription by RNA polymerase II GO:0045944 10.2 TGFB1 SMAD7 SMAD4 SMAD3 SMAD2 EDN1
3 positive regulation of transcription, DNA-templated GO:0045893 10.13 TGFB1 SPP1 SMAD4 SMAD3 SMAD2 EPO
4 negative regulation of transcription by RNA polymerase II GO:0000122 10.12 TGFB1 SMAD7 SMAD4 SMAD3 SMAD2 EPO
5 positive regulation of cell proliferation GO:0008284 10.11 TIMP1 TGFB1 EPO EDN1 CCN2
6 negative regulation of gene expression GO:0010629 10.03 TGFB1 SMAD2 EDN1 CCN2
7 protein deubiquitination GO:0016579 10.02 SMAD7 SMAD4 SMAD3 SMAD2
8 positive regulation of cell migration GO:0030335 10 TGFB1 SMAD3 EDN1 CCL7
9 heart development GO:0007507 9.99 TGFB1 SMAD2 EDNRA EDN1
10 cytokine-mediated signaling pathway GO:0019221 9.98 TIMP1 TGFB1 PIK3CA F13A1 CXCL2
11 positive regulation of ERK1 and ERK2 cascade GO:0070374 9.97 TGFB1 EPO CCN2 CCL7
12 aging GO:0007568 9.93 TIMP1 TGFB1 EPO DCN CCN2
13 in utero embryonic development GO:0001701 9.91 SMAD4 SMAD3 SMAD2 RCN1 EDNRA EDN1
14 cellular calcium ion homeostasis GO:0006874 9.9 TGFB1 EDN1 CCL7
15 anatomical structure morphogenesis GO:0009653 9.9 SMAD7 SMAD4 SMAD3 SMAD2
16 positive regulation of phosphatidylinositol 3-kinase signaling GO:0014068 9.89 PIK3CA EPO DCN
17 positive regulation of peptidyl-serine phosphorylation GO:0033138 9.89 TGFB1 PIK3CA MAPKAP1
18 response to organic substance GO:0010033 9.89 TIMP1 TGFB1 SPP1
19 transforming growth factor beta receptor signaling pathway GO:0007179 9.88 TGFB1 SMAD7 SMAD4 SMAD3 SMAD2
20 neutrophil chemotaxis GO:0030593 9.87 EDN1 CXCL2 CCL7
21 response to glucose GO:0009749 9.85 TGFB1 SMAD2 CCN2
22 negative regulation of transforming growth factor beta receptor signaling pathway GO:0030512 9.85 TGFB1 SMAD7 SMAD3 SMAD2
23 wound healing GO:0042060 9.85 TIMP1 TGFB1 SMAD3 SMAD2 DCN
24 cellular response to transforming growth factor beta stimulus GO:0071560 9.83 TGFB1 SMAD7 SMAD3 EDN1
25 positive regulation of epithelial to mesenchymal transition GO:0010718 9.81 TGFB1 SMAD4 SMAD3 SMAD2
26 ureteric bud development GO:0001657 9.8 TGFB1 SMAD7 SMAD3 SMAD2
27 response to hypoxia GO:0001666 9.8 TGFB1 SMAD4 SMAD3 EPO EDNRA EDN1
28 positive regulation of pri-miRNA transcription by RNA polymerase II GO:1902895 9.78 TGFB1 SMAD4 SMAD3
29 gastrulation GO:0007369 9.77 SMAD4 SMAD3 SMAD2
30 BMP signaling pathway GO:0030509 9.77 TGFB1 SMAD7 SMAD4 SMAD3 SMAD2
31 developmental growth GO:0048589 9.74 SMAD4 SMAD3 SMAD2
32 positive regulation of SMAD protein signal transduction GO:0060391 9.73 TGFB1 SMAD4
33 secondary palate development GO:0062009 9.72 SMAD4 SMAD2
34 pathway-restricted SMAD protein phosphorylation GO:0060389 9.72 TGFB1 SMAD7
35 response to cholesterol GO:0070723 9.71 TGFB1 SMAD2
36 lens fiber cell differentiation GO:0070306 9.71 TGFB1 SMAD3
37 endoderm development GO:0007492 9.71 TGFB1 SMAD4 SMAD3 SMAD2
38 cell activation GO:0001775 9.7 TIMP1 TGFB1
39 response to transforming growth factor beta GO:0071559 9.7 SMAD4 EDN1
40 primary miRNA processing GO:0031053 9.7 SMAD3 SMAD2
41 artery smooth muscle contraction GO:0014824 9.69 EDNRA EDN1
42 response to dexamethasone GO:0071548 9.68 EPO EDN1
43 nodal signaling pathway GO:0038092 9.68 SMAD3 SMAD2
44 pericardium development GO:0060039 9.68 SMAD3 SMAD2
45 embryonic foregut morphogenesis GO:0048617 9.67 SMAD3 SMAD2
46 response to laminar fluid shear stress GO:0034616 9.67 TGFB1 SMAD7
47 regulation of glucose transmembrane transport GO:0010827 9.66 EDNRA EDN1
48 endothelin receptor signaling pathway GO:0086100 9.66 EDNRA EDN1
49 regulation of striated muscle tissue development GO:0016202 9.65 TGFB1 SMAD3
50 SMAD protein signal transduction GO:0060395 9.65 TGFB1 SMAD7 SMAD4 SMAD3 SMAD2

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

# Name GO ID Score Top Affiliating Genes
1 protein binding GO:0005515 10.38 TIMP1 TGM2 TGFB1 SPP1 SMAD7 SMAD4
2 collagen binding GO:0005518 9.56 SMAD7 SMAD4 SMAD3 DCN
3 R-SMAD binding GO:0070412 9.54 SMAD4 SMAD3 SMAD2
4 transforming growth factor beta receptor binding GO:0005160 9.5 TGFB1 SMAD3 SMAD2
5 co-SMAD binding GO:0070410 9.46 SMAD3 SMAD2
6 protein-glutamine gamma-glutamyltransferase activity GO:0003810 9.43 TGM2 F13A1
7 type I transforming growth factor beta receptor binding GO:0034713 9.33 TGFB1 SMAD7 SMAD2
8 I-SMAD binding GO:0070411 9.26 SMAD7 SMAD4 SMAD3 SMAD2
9 cytokine activity GO:0005125 9.17 TIMP1 TGFB1 SPP1 EPO EDN1 CXCL2

Sources for Nephrogenic Systemic Fibrosis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 20-May-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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