2012年6月23日星期六

What Causes FSGS (Focal Segmental Glomerulonephritis)


Primary causes of FSGS (Focal Segmental Glomerulonephritis)
Primary causes of FSGS means that doctors have not found its underlying causes. Usually, it presents as Nephrotic Syndrome. However, there are several currently genetic reasons which are associated with FSGS.
● ACTN4. It encodes alpha-actinin 4. This protein crosslinks bundles of actin filaments and is present in the podocyte. Mutations in this protein associated with FSGS result in increased affinity for actin binding, formation of intracellular aggregates, and decreased protein half-life.
● TRPC6. It encodes a member of the canonical family of TRP channels. This family of ion channels conducts cations in a largely non-selective manner.
● CD2AP or CMS. The protein expressed by this gene is expressed in podocytes where it interacts with fyn and synaptopodin.
● APOL1. According to Nephrologists, these variants arose as a defensive mechanism againstTrypanosoma brucei rhodesiense or some other sub-Saharan parasite despite conferring high susceptibility to FSGS when inherited from both parents.
● INF2. It encodes a member of the formin family of actin-regulating proteins. The observation that alterations in this podocyte-expressed formin cause FSGS emphasizes the importance of fine regulation of actin polymerization in podocyte function.
● NPHS2. Mutations in the NPHS2 gene, which codes for the protein called podocin, can cause focal segmental glomerulosclerosis.
Secondary cause of FSGS (Focal Segmental Glomerulonephritis)
Secondary cause of FSGS means that it is associated with the other disorders.
● Infections, including HIV, Hepatitis B and Parvovirus.
● Diseases, such as massive obesity, sickle cell nephropathy and congenital cyanotic heart disease.
● The damage to glomeruli. There are thousands of tiny filters in kidneys, called glomeruli, which overworks to push their limits by other diseases.
No matter primary or secondary FSGS causes, it is sure that FSGS results from the damage of glomerular basement membrane. What we can do is to repair the impaired glomeruli, thus avoids or delay the progress of FSGS (Focal Segmental Glomerulonephrits)

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