Complement related kidney diseases: Recurrence after transplantation

Table 2 Risk of atypical hemolytic uremic syndrome recurrence according to the implicated genetic abnormality

Gene	Protein location	Functional impact	Mutation frequency in aHUS (%)	Recurrence frequency after transplantation (%)
Mutation
CFH	Plasma	Loss	20-30	75-90
CFI	Plasma	Loss	2-12	45-80
CFB	Plasma	Gain	1-2	100
C3	Plasma	Gain	5-10	40-70
MCP	Membrane	Loss	10-15	15-20
THBD	Membrane	Loss	5	1 case
Genetic polymorphism (frequency in control population)
Homozygous CFHR1del (3%-8%)	Circulating	Undetermined	14-23 (> 90% in patients with anti-CFH antibodies	NA

aHUS: Atypical hemolytic uremic syndrome; C3: Complement C3; CFH: Complement factor H; CFI: Complement factor I; CFB: Complement factor B; MCP: Membrane cofactor protein; THBD: Thrombomodulin; CFHR1: Complement factor H receptor 1; NA: Not available.