Details of Disease

General Information of Disease (ID: DISNHRNG)

Disease Name Immunoglobulin-mediated membranoproliferative glomerulonephritis
Synonyms
hemolytic uremic syndrome, atypical, susceptibility to, 7; Ahus, susceptibility to, 7; nephrotic syndrome, type 7, with membranoproliferative glomerulonephritis; NPHS7; nephrotic syndrome, type 7; immunoglobulin-mediated membranoproliferative glomerulonephritis; membranoproliferative glomerulonephritis type I; Ig-mediated membranoproliferative glomerulonephritis; Immunoglobulin-mediated MPGN; Ig-mediated MPGN; immune Complex mediated membranoproliferative glomerulonephritis; Mesangiocapillary glomerulonephritis type 1
Definition
Glomerulonephritis characterized by mesangial proliferation, endocapillary proliferation, and glomerular capillary wall remodeling with immune complex deposits from classical complement pathway activation.
Disease Hierarchy
DISW087P: Primary membranoproliferative glomerulonephritis
DISECBR1: Hereditary nephritis
DISADF8G: Familial nephrotic syndrome
DISNHRNG: Immunoglobulin-mediated membranoproliferative glomerulonephritis
Disease Identifiers
MONDO ID
MONDO_0014005
UMLS CUI
C3554330
OMIM ID
615008
MedGen ID
767244
Orphanet ID
329903

Molecular Interaction Atlas (MIA) of This Disease

Molecular Interaction Atlas (MIA)
This Disease Is Related to 1 DOT Molecule(s)
Gene Name DOT ID Evidence Level Mode of Inheritance REF
DGKE OTWS86AS Strong Autosomal recessive [1]
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References

1 DGKE variants cause a glomerular microangiopathy that mimics membranoproliferative GN. J Am Soc Nephrol. 2013 Feb;24(3):377-84. doi: 10.1681/ASN.2012090903. Epub 2012 Dec 28.