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. Author manuscript; available in PMC: 2016 Jan 1.
Published in final edited form as: Ann Neurol. 2014 Nov 27;77(1):100–113. doi: 10.1002/ana.24306

Table 4.

Subjects with multiple variants in ALS genes

Patient Type Variant 1 Variant 2 Variant 3 Possible model
Sporadic SOD1(p.D91A) SOD1(p.D91A) - Homozygous recessive
Sporadic SETX(p.I2547T) SETX(p.I2547T) - Homozygous recessive
Sporadic SETX(p.C1554G) SETX(p.R168Q) - Potential compound het
Familial SOD1(p.A5V) DAO(p.S345F) - Oligogenic
Familial SOD1(p.P67A) SETX(p.I2547T) - Oligogenic
Familial C9ORF72 DCTN1(p.I196V) - Oligogenic
Familial C9ORF72 SQSTM1(p.V153I) - Oligogenic
Familial C9ORF72 SETX(p.I2547T) - Oligogenic
Sporadic ANG(p.K41I) VAPB(p.M170I) - Oligogenic
Sporadic ATXN2(22/31) SQSTM1(p.K238E) - Oligogenic
Sporadic FUS(p.R485W) SETX(p.I2547T) - Oligogenic
Sporadic DCTN1(p.R1049Q) SETX(p.S323N) - Oligogenic
Sporadic FUS(p.P525L) ATXN2(23/31) - Oligogenic
Sporadic TARDBP(p.G287S) VAPB(p.M170I) - Oligogenic
Familial SOD1(p.G38R) ANG(p.P136L) DCTN1(p.T1249I) Oligogenic
Sporadic ATXN2(22/32) DCTN1(p.T1249I) SETX(p.M274V) Oligogenic
Sporadic TAF15(p.R408C) SETX(p.I2547T) SETX(p.T14I) Oligogenic, potential compound het
Sporadic SETX(p.C1554G) DCTN1(p.H1270Q) FIG4(p.M694V) Oligogenic