FGS
MCID: FCL008
MIFTS: 60

Focal Glomerulosclerosis (FGS) malady

Neuronal diseases category

Summaries for Focal Glomerulosclerosis

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42NIH Rare Diseases, 21Genetics Home Reference, 32MalaCards
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NIH Rare Diseases:42 Fg syndrome (fgs) is a genetic condition that affects many parts of the body and occurs almost exclusively in males. "fg" represents the surname initials of the first individuals diagnosed with the disorder. people with fgs frequently have intellectual disability ranging from mild to severe, hypotonia, constipation and/or anal anomalies, a distinctive facial appearance, broad thumbs and great toes, a large head compared to body size (relative macrocephaly), and abnormalities of the corpus callosum. medical problems including heart defects, seizures, undescended testicle, and an inguinal hernia have also been reported in some affected individuals. researchers have identified five regions of the x chromosome that are linked to fgs in affected families. the condition is called fgs1 when it is caused by a change (mutation) in a gene called med12. fgs2 is caused by mutations in the flna gene. the genes responsible for fgs3, fgs4, and fgs5 remain unknown. fgs is inherited in an x-linked recessive pattern. last updated: 1/11/2010

MalaCards: Focal Glomerulosclerosis, also known as fg syndrome, is related to nephrotic syndrome and proteinuria. An important gene associated with Focal Glomerulosclerosis is FABP1 (fatty acid binding protein 1, liver), and among its related pathways are FOXA2 and FOXA3 transcription factor networks and Lipoprotein metabolism. The drug cyclosporine and the compounds captopril and palmitate have been mentioned in the context of this disorder. Affiliated tissues include kidney, heart and endothelial, and related mouse phenotypes are renal/urinary system and liver/biliary system.

Genetics Home Reference:21 FG syndrome is a genetic condition that affects many parts of the body and occurs almost exclusively in males. "FG" represents the surname initials of the first family diagnosed with the disorder.

Aliases & Classifications for Focal Glomerulosclerosis

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Sources:
8Disease Ontology, 63Wikipedia, 42NIH Rare Diseases, 22GTR, 21Genetics Home Reference, 10DISEASES, 60UMLS, 34MeSH, 56SNOMED-CT, 39NCIt
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Anatomical: Neuronal diseases


Aliases & Descriptions:

focal glomerulosclerosis 8 60
fg syndrome 63 42 21 10 60
fgs 8 42 22 21
opitz-kaveggia syndrome 42 21
keller syndrome 42 21
fgs1 42 21
mental retardation, large head, imperforate anus, congenital hypotonia, and partial agenesis of the corpus callosum 21
mental retardation, large head, imperforate anus, congenital hypotonia, and partial agenesis of corpus callosum 42
focal glomerular sclerosis 8
oks 21


External Ids:

Disease Ontology8 DOID:3945
MeSH34 D005923
SNOMED-CT56 236403004, 25821008
NCIt39 C37308

Related Diseases for Focal Glomerulosclerosis

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17GeneCards, 18GeneDecks
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Graphical network of the top 20 diseases related to Focal Glomerulosclerosis:



Diseases related to focal glomerulosclerosis

Clinical Features for Focal Glomerulosclerosis

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Drugs & Therapeutics for Focal Glomerulosclerosis

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5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 60UMLS, 40NDF-RT
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Approved drugs:

Search CenterWatch for Focal Glomerulosclerosis

Drug clinical trials:

Search ClinicalTrials for Focal Glomerulosclerosis

Search NIH Clinical Center for Focal Glomerulosclerosis

Search CenterWatch for Focal Glomerulosclerosis

Inferred drug relations via UMLS60/NDF-RT40:

Genetic Tests for Focal Glomerulosclerosis

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22GTR
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Genetic tests related to Focal Glomerulosclerosis:

id Genetic test Affiliating Genes
1 Fg Syndrome22

Anatomical Context for Focal Glomerulosclerosis

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32MalaCards
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MalaCards organs/tissues related to Focal Glomerulosclerosis:

32
Kidney, Heart, Endothelial, Liver, Bone, Monocytes, Bone marrow, Thymus, Eye, Lung, Myeloid

Animal Models for Focal Glomerulosclerosis or affiliated genes

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36MGI
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MGI Mouse Phenotypes related to Focal Glomerulosclerosis:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053677.7ALB, ACE, CLCN5, REN, NPHS2
2MP:00053707.5FABP1, LPL, CLCN5, ACE, ALB
3MP:00053766.4ALB, NPHS2, FABP1, LPL, VLDLR, REN

Publications for Focal Glomerulosclerosis

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50PubMed
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Articles related to Focal Glomerulosclerosis:

(show top 50)    (show all 733)
idTitleAuthorsYear
1
A retrospective study of focal segmental glomerulosclerosis: clinical criteria can identify patients at high risk for recurrent disease after first renal transplantation. (23433074)
2013
2
Distinct Metabolic Profile of Primary Focal Segmental Glomerulosclerosis Revealed by NMR-Based Metabolomics. (24244321)
2013
3
Human umbilical mesenchymal stem cells attenuate the progression of focal segmental glomerulosclerosis. (23514668)
2013
4
A form of apolipoprotein a-I is found specifically in relapses of focal segmental glomerulosclerosis following transplantation. (23205849)
2013
5
TRPC6 gene variants in Czech adult patients with focal segmental glomerulosclerosis and minimal change disease. (22980509)
2012
6
Histological heterogeneity of glomerular segmental lesions in focal segmental glomerulosclerosis. (21424374)
2012
7
Outcome of kidney allograft in patients with adulthood-onset focal segmental glomerulosclerosis: comparison with childhood-onset FSGS. (22187318)
2012
8
Regional secondary focal segmental glomerulosclerosis in a transplanted kidney: resolution with treatment of a segmental renal artery stenosis. (22691608)
2012
9
Immunosuppressive treatment of focal segmental glomerulosclerosis: lessons from a randomized controlled trial. (21960168)
2011
10
Role of intrarenal angiotensin system activation, oxidative stress, inflammation, and impaired nuclear factor-erythroid-2-related factor 2 activity in the progression of focal glomerulosclerosis. (21357516)
2011
11
Distinct metalloproteinase excretion patterns in focal segmental glomerulosclerosis. (21720805)
2011
12
Recurrence of focal segmental glomerulosclerosis in renal allograft: an in-depth review. (20519016)
2010
13
Focal segmental glomerulosclerosis in a female patient with Donnai-Barrow syndrome. (19952924)
2010
14
ACTN4 gene mutations and single nucleotide polymorphisms in idiopathic focal segmental glomerulosclerosis. (19142020)
2009
15
Treatment of focal segmental glomerulosclerosis with immunophilin modulation: when did we stop thinking about pathogenesis? (19494796)
2009
16
Low-density lipoprotein apheresis in a pediatric patient with refractory nephrotic syndrome due to focal segmental glomerulosclerosis. (19444573)
2009
17
Rituximab for refractory focal segmental glomerulosclerosis. (17973121)
2008
18
Successful treatment of focal segmental glomerulosclerosis in association with mantle cell lymphoma. (17497453)
2007
19
Educational feature on focal segmental glomerulosclerosis (FSGS): an introduction. (17106687)
2007
20
Immunoadsorbtion and rituximab therapy in a second living-related kidney transplant patient with recurrent focal segmental glomerulosclerosis. (17887957)
2007
21
Successful treatment of recurrent focal segmental glomerulosclerosis after kidney transplantation by plasmapheresis and rituximab. (17181660)
2007
22
Pathophysiology of minimal change nephrotic syndrome and focal segmental glomerulosclerosis. (17995521)
2007
23
Association of steroid and cyclosporin resistance in focal segmental glomerulosclerosis. (17294224)
2007
24
Laser capture microdissection-microarray analysis of focal segmental glomerulosclerosis glomeruli. (17684420)
2007
25
Recurrence of focal segmental glomerulosclerosis after renal transplantation: a case report. (17237899)
2007
26
Computational simulation of renal biopsy accuracy in focal segmental glomerulosclerosis. (16773406)
2006
27
Reduced podocyte expression of alpha3beta1 integrins and podocyte depletion in patients with primary focal segmental glomerulosclerosis and chronic PAN-treated rats. (16887498)
2006
28
Reduced podocyte expression of alpha3beta1 integrins and podocyte depletion in patients with primary focal segmental glomerulosclerosis and chronic PAN-treated rats. (16459165)
2006
29
Cellular focal segmental glomerulosclerosis: Clinical and pathologic features. (17021605)
2006
30
Increase in extracellular cross-linking by tissue transglutaminase and reduction in expression of MMP-9 contribute differentially to focal segmental glomerulosclerosis in rats. (16477388)
2006
31
Dual blockade of renin-angiotensin system in primary focal segmental glomerulosclerosis. (16278255)
2006
32
Renovascular hypertension: a unique cause of unilateral focal segmental glomerulosclerosis. (16755156)
2006
33
Focal segmental glomerulosclerosis in kidney resected for renal cell carcinoma. (16142569)
2005
34
Mutational and Biological Analysis of alpha-actinin-4 in focal segmental glomerulosclerosis. (16251236)
2005
35
Increased glomerular and extracellular malondialdehyde levels in patients and rats with focal segmental glomerulosclerosis. (15816993)
2005
36
Mycophenolate mofetil in the treatment of focal segmental glomerulosclerosis. (15630898)
2004
37
Focal segmental glomerulosclerosis in a girl with myelodysplastic syndrome. (15388820)
2004
38
Glomerular albumin permeability as an in vitro model for characterizing the mechanism of focal glomerulosclerosis and predicting post-transplant recurrence. (15265158)
2004
39
Effects of the genetic polymorphisms of the renin-angiotensin system on focal segmental glomerulosclerosis. (14610337)
2003
40
Protein restriction and AST-120 improve lipoprotein lipase and VLDL receptor in focal glomerulosclerosis. (14531811)
2003
41
Interferon-alpha-induced focal segmental glomerulosclerosis in chronic myelogenous leukemia: a case report and review of the literature. (12796597)
2003
42
Vascular permeability factors in steroid-sensitive nephrotic syndrome and focal segmental glomerulosclerosis. (12953037)
2003
43
Methylenetetrahydrofolate reductase polymorphism in childhood primary focal segmental glomerulosclerosis. (12218328)
2002
44
The new molecular revelations of recurrent focal segmental glomerulosclerosis in renal allografts. (17660677)
2002
45
Caught flat-footed: podocyte damage and the molecular bases of focal glomerulosclerosis. (11733553)
2001
46
A case of craniomandibular dermatodysostosis associated with focal glomerulosclerosis. (7632532)
1995
47
A contribution to nephrotic syndrome and focal glomerulosclerosis in a patient with adult polycystic kidney disease. (8321370)
1993
48
Glomerular size and the association of focal glomerulosclerosis in long-surviving human renal allografts. (8130358)
1993
49
Monocytes and macrophages in focal glomerulosclerosis in Zucker rats. (1944726)
1991
50
Altered expression of B lymphocyte surface immunoglobulins in minimal change nephrotic syndrome and focal glomerulosclerosis. (6611510)
1984

Genetic Variations for Focal Glomerulosclerosis

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Expression for genes affiliated with Focal Glomerulosclerosis

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1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Focal Glomerulosclerosis

Search GEO for disease gene expression data for Focal Glomerulosclerosis.

Pathways for genes affiliated with Focal Glomerulosclerosis

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37NCBI BioSystems Database, 53Reactome, 49PharmGKB
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Pathways related to Focal Glomerulosclerosis according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.5ALB, TAT
2
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9.4ALB, LPL
3
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9.0ACE, REN

Compounds for genes affiliated with Focal Glomerulosclerosis

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44Novoseek, 28IUPHAR, 49PharmGKB, 59Tocris Bioscience, 11DrugBank, 24HMDB
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Compounds related to Focal Glomerulosclerosis according to GeneCards/GeneDecks:

(show top 50)    (show all 73)
idCompoundScoreTop Affiliating Genes
1captopril44 28 49 59 1114.2REN
2palmitate4410.1FABP1
3norepinephrine44 11 2411.9LPL, TAT
4ornipressin449.7REN, ALB
551-cr-edta449.7REN, ALB
6terlipressin44 1110.6REN, ALB
7ibopamine449.4REN, ACE
8sampatrilat449.4ACE, REN
9remikiren44 1110.4ACE, REN
10perindoprilat449.4ACE, REN
11alacepril449.4REN, ACE
12aliskiren44 1110.4ACE, REN
13oleic acid44 28 11 2412.4ALB, LPL, FABP1
14bucindolol49 4410.4ACE, REN
15dihydralazine449.3ACE, REN
16lacidipine449.3ACE, REN
17zidovudine44 1110.3TAT, LPL, ALB
18saralasin449.3ACE, REN
19benazepril44 1110.3ACE, REN
20eprosartan44 28 1111.2ACE, REN
21trandolapril44 1110.2REN, ACE
22cilazapril44 1110.2REN, ACE
23phospholipid449.2VLDLR, LPL, TAT, FABP1
24omapatrilat449.1ACE, REN
25bisoprolol44 49 1111.1ACE, REN
26inulin449.0ALB, REN
27eplerenone44 59 28 1112.0ACE, REN
28indapamide44 1110.0ACE, REN, ALB
29fosinopril44 1110.0REN, ALB, ACE
30lisinopril44 11 2410.9ACE, ALB, REN
31ramipril44 119.9ACE, ALB, REN
32valsartan44 59 28 1111.9REN, ALB, ACE
33thiazide448.9ACE, REN, LPL
34telmisartan44 28 1110.9ALB, ACE, REN
35perindopril44 49 1110.9ALB, ACE, REN
36irbesartan44 28 49 1111.9ALB, ACE, REN
37hydrochlorothiazide44 49 28 11 2412.9ALB, ACE, REN
38candesartan44 28 1110.9ALB, ACE, REN
39enalapril44 49 1110.9ACE, ALB, REN
40spironolactone49 44 59 28 1112.9REN, ACE, ALB
41amlodipine44 49 11 2411.9REN, ACE, ALB
42furosemide44 49 59 28 11 2413.9REN, ALB, ACE
43kininogen448.9REN, ACE, ALB
44losartan44 49 28 1111.8ALB, ACE, REN
45indomethacin44 59 28 1111.8REN, TAT, LPL, ALB
46fatty acid448.7FABP1, LPL, VLDLR, ALB
47cholesterol44 28 11 2410.7VLDLR, LPL, ALB, ACE, FABP1, REN
48glucose447.7ALB, ACE, TAT, REN, LPL, FABP1
49lipid447.6FABP1, ACE, TAT, VLDLR, NPHS2, LPL
50creatinine447.5ALB, ACE, CLCN5, REN, VLDLR, FABP1

GO Terms for genes affiliated with Focal Glomerulosclerosis

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16Gene Ontology
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Cellular components related to Focal Glomerulosclerosis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1very-low-density lipoprotein particleGO:0343619.3VLDLR, LPL
2extracellular spaceGO:0056158.0ALB, ACE, REN, LPL

Biological processes related to Focal Glomerulosclerosis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1excretionGO:0075889.7CLCN5, NPHS2
2response to mercury ionGO:0466899.5ALB, TAT
3lipoprotein metabolic processGO:0421579.1ALB, LPL
4angiotensin maturationGO:0020038.9ACE, REN

Molecular functions related to Focal Glomerulosclerosis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1antioxidant activityGO:0162099.6ALB, FABP1
2apolipoprotein bindingGO:0341859.5VLDLR, LPL
3fatty acid bindingGO:0055049.3ALB, FABP1
4drug bindingGO:0081448.8ALB, ACE, FABP1

Products for genes affiliated with Focal Glomerulosclerosis

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Sources for Focal Glomerulosclerosis

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet