Phenotypes associated with this allele

• Allele Symbol: Cfi^tm1Mcp
• Allele Name: targeted mutation 1, Matthew C Pickering
• Allele ID: MGI:3773693
• Summary: 3 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
hm1	Cfi^tm1Mcp/Cfi^tm1Mcp	involves: 129/Sv * C57BL/6	MGI:3778084
cx2	Cfh^tm1Mbo/Cfh^+ Cfi^tm1Mcp/Cfi^tm1Mcp	involves: 129/Sv * C57BL/6	MGI:3778085
cx3	Cfh^tm1Mbo/Cfh^tm1Mbo Cfi^tm1Mcp/Cfi^tm1Mcp	involves: 129/Sv * C57BL/6	MGI:3778086

Genotype
MGI:3778084

hm1

• Allelic Composition: Cfi^tm1Mcp/Cfi^tm1Mcp
• Genetic Background: involves: 129/Sv * C57BL/6

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

immune system

decreased circulating complement protein level ( J:131403 )

• median plasma level of complement protein C3 is significantly lower than in heterozygotes or wild-type animals

• complement factor H and factor B levels are significantly reduced

• no cleavage of C3b is detected, as indicated by absence of C3 alpha-chain fragments in plasma

renal/urinary system

abnormal renal glomerulus morphology ( J:131403 )

• glomerular hypercellularity, and capillary wall thickening are observed in kidneys at 8 months; these are similar to controls

• deposition of nodular hyaline material within the mesangium is observed in close to half of the animals

expanded mesangial matrix ( J:131403 )

• significant mesangial expansion is seen compared to controls

renal glomerular protein deposits ( J:131403 )

• C3 deposition along glomerulus basement membrane (GBM) is significantly increased relative to controls; pattern is mesangial distribution compared to the linear capillary wall deposition pattern observed in Cfh-deficient mice

homeostasis/metabolism

decreased circulating complement protein level ( J:131403 )

• median plasma level of complement protein C3 is significantly lower than in heterozygotes or wild-type animals

• complement factor H and factor B levels are significantly reduced

• no cleavage of C3b is detected, as indicated by absence of C3 alpha-chain fragments in plasma

Genotype
MGI:3778085

cx2

• Allelic Composition: Cfh^tm1Mbo/Cfh⁺; Cfi^tm1Mcp/Cfi^tm1Mcp
• Genetic Background: involves: 129/Sv * C57BL/6

renal/urinary system

renal glomerular protein deposits ( J:131403 )

• C3 deposition along glomerulus basement membrane (GBM) is identical to that observed in Cfi-deficient kidneys (significantly increased relative to controls)

Genotype
MGI:3778086

cx3

• Allelic Composition: Cfh^tm1Mbo/Cfh^tm1Mbo; Cfi^tm1Mcp/Cfi^tm1Mcp
• Genetic Background: involves: 129/Sv * C57BL/6

renal/urinary system

renal/urinary system phenotype ( J:131403 )

N • no membranoproliferative glomerulonephritis type II (MPGN2) is observed at 8 months compared with Cfh-null animals

renal glomerular protein deposits ( J:131403 )

• C3 deposition along glomerulus basement membrane (GBM) is identical to that observed in Cfh-deficient kidneys (significantly increased relative to controls)

homeostasis/metabolism

abnormal circulating protein level ( J:131403 )

• median plasma level of complement protein C3 is significantly higher than Cfh^tm1Mbo-deficient animals and is similar to Cfi-null mice

• no cleavage of C3 is detected in plasma