Laboratory for Molecular Diagnostics
Center for Nephrology and Metabolic Disorders

Membranoproliferative glomerulonephritis (MPGN)

MPGN is a rare type of glomerulonephritis with typical immunohistochemical findings. This histomorphological classification is almost abandoned in favor of the more pathophysiologically oriented classification as C3 glomerulopathy in which the abnormal activation of the alternative complement pathway plays a crucial role. In connection with such pathophysiological considerations molecular genetic diagnosis gains importance.

Classification

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Membranoproliferative glomerulonephritis (MPGN) I

Discrete immune complex deposition in the mesangium and subendothelial space with secondary activation of the classical complement pathway.

Membranoproliferative glomerulonephritis (MPGN) II

Continuous, electron-dense, ribbon-like, C3-rich deposition along glomerular basement membrane, tubular basement membrane, and Bowman's capsule. The cause is an over-activation of the alternative complement pathway.

Membranoproliferative glomerulonephritis (MPGN) III

Burkholder variant: Multiple subepithelial and subendothelial deposits.
Strife and Anders variant: Predominantly intramembranous deposits and disruption of the glomerular basement membrane.

Systematic

Glomerulonephritis
C3 glomerulopathy
CFHR5 Nephropathy
Goodpasture syndrome
Lupus erythematosus nephritis
Membranoproliferative glomerulonephritis (MPGN)
ADAMTS13
C1QA
C1QB
C1QC
C3
CD46
CFB
CFD
CFH
CFHR1
CFHR2
CFHR3
CFHR4
CFHR5
CFI
CLU
CR1 deficiency
CR1
Complement component C1q deficiency
C1QA
C1QB
C1QC
Complement component C1s deficiency
C1S
DGKE
PIGA
THBD
Membranous nephropathy
Mesangioproliferative glomerulonephritis

References:

1.

Sánchez-Corral P et al. (2000) Molecular basis for factor H and FHL-1 deficiency in an Italian family.

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2.

Ault BH et al. (2000) Factor H and the pathogenesis of renal diseases.

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3.

Pickering MC et al. (2002) Uncontrolled C3 activation causes membranoproliferative glomerulonephritis in mice deficient in complement factor H.

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4.

Hegasy GA et al. (2002) The molecular basis for hereditary porcine membranoproliferative glomerulonephritis type II: point mutations in the factor H coding sequence block protein secretion.

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5.

Dragon-Durey MA et al. (2004) Heterozygous and homozygous factor h deficiencies associated with hemolytic uremic syndrome or membranoproliferative glomerulonephritis: report and genetic analysis of 16 cases.

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6.

Saunders RE et al. (2006) An interactive web database of factor H-associated hemolytic uremic syndrome mutations: insights into the structural consequences of disease-associated mutations.

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7.

Abrera-Abeleda MA et al. (2006) Variations in the complement regulatory genes factor H (CFH) and factor H related 5 (CFHR5) are associated with membranoproliferative glomerulonephritis type II (dense deposit disease).

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8.

Licht C et al. (2006) Deletion of Lys224 in regulatory domain 4 of Factor H reveals a novel pathomechanism for dense deposit disease (MPGN II).

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9.

Servais A et al. (2007) Primary glomerulonephritis with isolated C3 deposits: a new entity which shares common genetic risk factors with haemolytic uraemic syndrome.

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10.

Levy M et al. (1986) H deficiency in two brothers with atypical dense intramembranous deposit disease.

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11.

Brai M et al. (1988) Combined homozygous factor H and heterozygous C2 deficiency in an Italian family.

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12.

Vogt BA et al. (1995) Inherited factor H deficiency and collagen type III glomerulopathy.

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13.

Høgåsen K et al. (1995) Hereditary porcine membranoproliferative glomerulonephritis type II is caused by factor H deficiency.

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14.

Ault BH et al. (1997) Human factor H deficiency. Mutations in framework cysteine residues and block in H protein secretion and intracellular catabolism.

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15.

Rougier N et al. (1998) Human complement factor H deficiency associated with hemolytic uremic syndrome.

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16.

McRae JL et al. (2002) Location and structure of the human FHR-5 gene.

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17.

McRae JL et al. (2001) Human factor H-related protein 5 (FHR-5). A new complement-associated protein.

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18.

Wyatt RJ et al. (1982) Partial H (beta 1H) deficiency and glomerulonephritis in two families.

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19.

Fijen CA et al. (1996) Heterozygous and homozygous factor H deficiency states in a Dutch family.

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20.

Welch TR et al. (2002) Complement in glomerulonephritis.

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21.

Appel GB et al. (2005) Membranoproliferative glomerulonephritis type II (dense deposit disease): an update.

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22.

Strife CF et al. (1977) Membranoproliferative glomerulonephritis with disruption of the glomerular basement membrane.

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23.

Stutchfield PR et al. (1986) X-linked mesangiocapillary glomerulonephritis.

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24.

Habib R et al. () Idiopathic membranoproliferative glomerulonephritis in children. Report of 105 cases.

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25.

Mandalenakis N et al. (1971) Lobular glomerulonephritis and membranoproliferative glomerulonephritis: a clinical and pathologic study based on renal biopsies.

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26.

Berry PL et al. (1981) Membranoproliferative glomerulonephritis in two sibships.

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Update: Sept. 26, 2018