FGS
MCID: FCL008
MIFTS: 57

Focal Glomerulosclerosis (FGS) malady

Neuronal category

Summaries for Focal Glomerulosclerosis

Sources:
21Genetics Home Reference, 43NIH Rare Diseases, 33MalaCards
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NIH Rare Diseases:43 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 focal segmental glomerulosclerosis and nephrosis. 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 liver, kidney and lung, 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

Sources:
64Wikipedia, 43NIH Rare Diseases, 21Genetics Home Reference, 10DISEASES, 61UMLS, 8Disease Ontology, 22GTR, 35MeSH, 57SNOMED-CT, 40NCIt
See all sources

Classifications:

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


Aliases & Descriptions:

focal glomerulosclerosis 8 61
fg syndrome 64 43 21 10 61
fgs 8 43 22 21
opitz-kaveggia syndrome 43 21
keller syndrome 43 21
fgs1 43 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 43
focal glomerular sclerosis 8
oks 21


External Ids:

Disease Ontology8 DOID:3945
MeSH35 D005923
SNOMED-CT57 236403004, 25821008
NCIt40 C37308

Related Diseases for Focal Glomerulosclerosis

Sources:
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

Drugs & Therapeutics for Focal Glomerulosclerosis

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials, 61UMLS, 41NDF-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 UMLS61/NDF-RT41:

Genetic Tests for Focal Glomerulosclerosis

Sources:
22GTR
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Genetic tests related to Focal Glomerulosclerosis:

id Genetic test Affiliating Genes
1 Fg Syndrome22

Anatomical Context for Focal Glomerulosclerosis

Sources:
33MalaCards
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MalaCards organs/tissues related to Focal Glomerulosclerosis:

33
Liver, Kidney, Lung, Bone marrow, Thymus, Heart, Myeloid, Monocytes, B cells, Endothelial

Animal Models for Focal Glomerulosclerosis or affiliated genes

Sources:
37MGI, 28inGenious Targeting Laboratory
See all sources

MGI Mouse Phenotypes related to Focal Glomerulosclerosis:

37
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

Sources:
51PubMed
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Articles related to Focal Glomerulosclerosis:

(show top 50)    (show all 734)
idTitleAuthorsYear
1
The direction and role of phenotypic transition between podocytes and parietal epithelial cells in focal segmental glomerulosclerosis. (24154691)
2014
2
Serum-soluble urokinase receptor levels do not distinguish focal segmental glomerulosclerosis from other causes of nephrotic syndrome in children. (24429405)
2014
3
Mutational analysis of ACTN4, encoding I+-actinin 4, in patients with focal segmental glomerulosclerosis using HRM method. (23890478)
2013
4
Early focal segmental glomerulosclerosis as a cause of renal allograft primary nonfunction. (23781382)
2013
5
Pathobiology of focal segmental glomerulosclerosis: new developments. (22357339)
2012
6
An explorative analysis of secretory receptor for advanced glycation endproducts in primary focal segmental glomerulosclerosis. (22302086)
2012
7
Therapeutic apheresis rescue mission: recurrent focal segmental glomerulosclerosis in renal allografts. (22175233)
2012
8
Collapsing focal segmental glomerulosclerosis in a young child. (23128477)
2012
9
Circulating mediators of focal segmental glomerulosclerosis: soluble urokinase plasminogen activator receptor in context. (22033283)
2012
10
A case of de novo focal segmental glomerulosclerosis occurred one and half years after kidney transplantation supposed to be caused by calcineurin inhibitor. (22747481)
2012
11
Treatment of recurrent focal segmental glomerulosclerosis in pediatric kidney transplant recipients: effect of rituximab. (21577271)
2011
12
MYO1E, focal segmental glomerulosclerosis, and the cytoskeleton. (21751899)
2011
13
Coincident idiopathic focal segmental glomerulosclerosis collapsing variant and diabetic nephropathy in an African American homozygous for MYH9 risk variants. (21074826)
2011
14
Adriamycin nephropathy: a model of focal segmental glomerulosclerosis. (21175974)
2011
15
Clinical course of primary focal segmental glomerulosclerosis (FSGS) in Turkish children: a report from the Turkish Pediatric Nephrology FSGS Study Group. (20718182)
2010
16
Rituximab in minimal change nephropathy and focal segmental glomerulosclerosis: report of four cases and review of the literature. (19011266)
2008
17
Focal segmental glomerulosclerosis and nephrotic syndrome in a child with embryonal rhabdomyosarcoma. (18175060)
2008
18
Crystal structure of the actin-binding domain of alpha-actinin-4 Lys255Glu mutant implicated in focal segmental glomerulosclerosis. (18164029)
2008
19
Functional genetic variation in aminopeptidase A (ENPEP): lack of clear association with focal and segmental glomerulosclerosis (FSGS). (18206321)
2008
20
Galactose binds to focal segmental glomerulosclerosis permeability factor and inhibits its activity. (18514139)
2008
21
Simultaneous and sustained remission of intractable myasthenia gravis and focal segmental glomerulosclerosis with tacrolimus treatment. (18793550)
2008
22
Podocyte injury in focal segmental glomerulosclerosis: Lessons from animal models (a play in five acts). (17989648)
2008
23
Rituximab failed to improve nephrotic syndrome in renal transplant patients with recurrent focal segmental glomerulosclerosis. (17979998)
2008
24
NPHS2 variation in sporadic focal segmental glomerulosclerosis. (17942957)
2007
25
Recurrent autosomal-dominant focal segmental glomerulosclerosis. (17051266)
2006
26
Strumpell's disease in a family with hereditary focal segmental glomerulosclerosis. (16771252)
2006
27
Focal segmental glomerulosclerosis associated with essential thrombocythemia. (16544181)
2006
28
A drug target for focal segmental glomerulosclerosis. (15939909)
2005
29
Four-month-old infant with focal segmental glomerulosclerosis and mitochondrial DNA deletion. (15791930)
2005
30
Effects of steroids in focal segmental glomerulosclerosis in a predominantly African-American population. (16020995)
2005
31
Glomerular tip lesion: a distinct entity within the minimal change disease/focal segmental glomerulosclerosis spectrum. (15086908)
2004
32
Focal segmental glomerulosclerosis in solitary kidney in WAGR syndrome. (15354948)
2004
33
Recurrent focal glomerulosclerosis in the era of genetics of podocyte proteins: theory and therapy. (15004256)
2004
34
Nephrotic focal segmental glomerulosclerosis in 2004: an update. (15280528)
2004
35
Efficacy of losartan in patients with primary focal segmental glomerulosclerosis resistant to immunosuppressive treatment. (12603500)
2003
36
Glomerular endothelial cytotoxicity and dysfunction in nephrosis with focal segmental glomerulosclerosis. (14724375)
2003
37
Focal segmental glomerulosclerosis in a patient with large bilateral asymptomatic adrenal myelolipomas. (14669865)
2003
38
Another autosomal recessive form of focal glomerulosclerosis with neurological findings. (11793129)
2002
39
Inhibition of renal permeability towards albumin: a new function of apolipoproteins with possible pathogenetic relevance in focal glomerulosclerosis. (11425237)
2001
40
Prevalence, genetics, and clinical features of patients carrying podocin mutations in steroid-resistant nonfamilial focal segmental glomerulosclerosis. (11729243)
2001
41
Mutations in ACTN4, encoding alpha-actinin-4, cause familial focal segmental glomerulosclerosis. (10700177)
2000
42
Phenotypic characteristics and cyclin-dependent kinase inhibitors repression in hyperplastic epithelial pathology in idiopathic focal segmental glomerulosclerosis. (10879738)
2000
43
The long-term prognosis of benign nephrosclerosis accompanied by focal glomerulosclerosis and renal cortical interstitial fibrosis, designated so-called decompensated benign nephrosclerosis by Fahr, Bohle and Ratscheck. (9779492)
1998
44
Familial focal glomerulosclerosis: a genetic linkage to the HLA locus? (7503147)
1995
45
Glomerular dysfunction in nephrotic humans with minimal changes or focal glomerulosclerosis. (1709791)
1991
46
Rapid renal failure in AIDS-associated focal glomerulosclerosis. (2405802)
1990
47
Disappearance of proteinuria after immunoadsorption in a patient with focal glomerulosclerosis. (1973517)
1990
48
Focal glomerulosclerosis treated with heparin. (2774618)
1989
49
The composition of glomerulosclerosis. I. Studies in focal sclerosis, crescentic glomerulonephritis, and membranoproliferative glomerulonephritis. (6748613)
1984
50
Prognosis of focal glomerulosclerosis. (448521)
1979

Genetic Variations for Focal Glomerulosclerosis

Expression for genes affiliated with Focal Glomerulosclerosis

Sources:
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

Sources:
38NCBI BioSystems Database, 54Reactome, 50PharmGKB
See all sources

Pathways related to Focal Glomerulosclerosis according to GeneCards/GeneDecks:

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

Compounds for genes affiliated with Focal Glomerulosclerosis

Sources:
45Novoseek, 29IUPHAR, 50PharmGKB, 60Tocris 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
1captopril45 29 50 60 1114.2REN
2palmitate4510.1FABP1
3norepinephrine45 11 2411.9LPL, TAT
4ornipressin459.7ALB, REN
551-cr-edta459.7REN, ALB
6terlipressin45 1110.6REN, ALB
7remikiren45 1110.4ACE, REN
8sampatrilat459.4ACE, REN
9ibopamine459.4ACE, REN
10alacepril459.4ACE, REN
11perindoprilat459.4REN, ACE
12oleic acid45 29 11 2412.4ALB, LPL, FABP1
13aliskiren45 1110.4REN, ACE
14dihydralazine459.4ACE, REN
15bucindolol50 4510.3ACE, REN
16lacidipine459.3ACE, REN
17zidovudine45 1110.3LPL, ALB, TAT
18saralasin459.3ACE, REN
19eprosartan45 29 1111.3REN, ACE
20benazepril45 1110.2REN, ACE
21trandolapril45 1110.2REN, ACE
22cilazapril45 1110.2ACE, REN
23phospholipid459.2TAT, FABP1, VLDLR, LPL
24omapatrilat459.1ACE, REN
25bisoprolol45 50 1111.1ACE, REN
26inulin459.0REN, ALB
27olmesartan45 29 1111.0REN, ACE
28indapamide45 1110.0ACE, REN, ALB
29fosinopril45 1110.0REN, ALB, ACE
30lisinopril45 11 2410.9ACE, ALB, REN
31ramipril45 119.9ACE, ALB, REN
32valsartan45 60 29 1111.9REN, ALB, ACE
33thiazide458.9ACE, REN, LPL
34telmisartan45 29 1110.9ALB, ACE, REN
35perindopril45 50 1110.9ALB, ACE, REN
36irbesartan45 29 50 1111.9ALB, ACE, REN
37hydrochlorothiazide45 50 29 11 2412.9ALB, ACE, REN
38candesartan45 29 1110.9ALB, ACE, REN
39enalapril45 50 1110.9ACE, ALB, REN
40spironolactone50 45 60 29 1112.9REN, ACE, ALB
41amlodipine45 50 11 2411.9ALB, REN, ACE
42furosemide45 50 60 29 11 2413.9REN, ACE, ALB
43kininogen458.9ALB, ACE, REN
44losartan45 50 29 1111.8REN, ALB, ACE
45indomethacin45 60 29 1111.8ALB, REN, TAT, LPL
46fatty acid458.7VLDLR, ALB, FABP1, LPL
47cholesterol45 29 11 2410.7VLDLR, LPL, ALB, ACE, FABP1, REN
48glucose457.7ALB, ACE, TAT, REN, LPL, FABP1
49lipid457.6FABP1, ACE, TAT, VLDLR, NPHS2, LPL
50creatinine457.5ALB, ACE, CLCN5, REN, VLDLR, FABP1

GO Terms for genes affiliated with Focal Glomerulosclerosis

Sources:
16Gene Ontology
See all sources

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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  • Proteins
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  • Antibodies

Sources for Focal Glomerulosclerosis

3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet