FB2026_01 , released March 12, 2026
FB2026_01 , released March 12, 2026
Human Disease Model Report: focal segmental glomerulosclerosis
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General Information
Name
focal segmental glomerulosclerosis
FlyBase ID
FBhh0000617
Disease Ontology Term
Parent Disease
OMIM
Overview

This report describes general characteristics of the group of diseases classified as focal segmental glomerulosclerosis (FSGS). FSGS is a genetically heterogeneous disorder with multiple causative genes and mapped loci. A comprehensive list of FSGS subtypes, as defined by OMIM can be found by following the link in the "OMIM phenotypic series" section, below. A subset of these are listed in the table below, with links to more detailed reports for subtypes that have been investigated using fly models.

In the literature, the clinical term 'nephrotic syndrome' (NPHS) and the pathologic term 'focal segmental glomerulosclerosis' (FSGS) have often been used to refer to the same disease entity; see the human disease model report 'nephrotic syndrome', FBhh0000317.

[updated Sep. 2017 by FlyBase; FBrf0222196]

Disease Summary Information
Parent Disease Summary: focal segmental glomerulosclerosis
OMIM report
Symptoms and phenotype

Focal segmental glomerulosclerosis is one of many diseases and conditions can affect kidney function by attacking and damaging the glomeruli. "Glomerulosclerosis" refers to a hardening and scarring of the glomeruli. The scarring of FSGS takes place in small sections of each glomerulus, and only a limited number of glomeruli are damaged initially (https://www.kidney.org/atoz/content/focal).

(FlyBase Curators, 2013-)

A definitive diagnosis of FSGS is established only by histopathology findings (http://emedicine.medscape.com/article/245915-overview).

(FlyBase Curators, 2013-)

Focal segmental glomerulosclerosis is a common cause of end-stage renal disease (Meyrier, 2005; pubmed:16932363). [from MIM:607832; 2017.09.14]

(OMIM, 2013-)

In the literature, the clinical term 'nephrotic syndrome' (NPHS) and the pathologic term 'focal segmental glomerulosclerosis' (FSGS) have often been used to refer to the same disease entity. In OMIM, these disorders are classified as NPHS or FSGS according to how they were first designated in the literature. [from MIM:607832; 2017.09.14]

(OMIM, 2013-)

Focal segmental glomerulosclerosis (FSGS) is a pathologic finding in several renal disorders that manifest clinically as proteinuria and progressive decline in renal function. Some patients with FSGS develop nephrotic syndrome, which includes massive proteinuria, hypoalbuminemia, hyperlipidemia, and edema. However, patients with FSGS may have proteinuria in the nephrotic range without other features of the nephrotic syndrome (summary by D'Agati et al., 2004, pubmed:14750104; Mathis et al., 1998, pubmed:9461087). [from MIM:603278; 2017.09.14]

(OMIM, 2013-)
Genetics
Cellular phenotype and pathology
Molecular information
External links
    Disease synonyms
    focal glomerular sclerosis
    focal nodular glomerulosclerosis
    FSGS
    Ortholog Information
    Human gene(s) in FlyBase
      Other mammalian ortholog(s) used
        D. melanogaster Gene Information (0)
        Other Genes Used: Viral, Bacterial, Synthetic (0)
          Summary of Physical Interactions (0 groups)
          Alleles Reported to Model Human Disease (Disease Ontology) (0 alleles)
          Alleles Representing Disease-Implicated Variants
          Genetic Tools, Stocks and Reagents
          Sources of Stocks
          Contact lab of origin for a reagent not available from a public stock center.
          Bloomington Stock Center Disease Page
          Related mammalian, viral, bacterial, or synthetic transgenes
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          Publicly Available Stocks
          Selected Drosophila transgenes
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          Publicly Available Stocks
          RNAi constructs available
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          Selected Drosophila classical alleles
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          Publicly Available Stocks
          References (2)