Table 2.

MUNC13-4 mutations in patients with FHL3


UPN, mutations

Mutation type

Predicted effect

Munc13-4 expression
180    Absent  
    G175A   Heterozygous   A59T   
    753 + 1G > T   Homozygous   splice error   
277    Trace  
    del 3082C   Heterozygous   1028 Fs   
    G1241T, C2782T   Heterozygous   R414L, R928C   
249    Trace  
    A1847G   Homozygous   E616G   
237    Trace  
    A1847G   Heterozygous   E616G   
    G1241T, C2782T   Heterozygous   R414L, R928C   
225    Trace  
    A1847G   Heterozygous   E616G   
    753 + 1G > T   Heterozygous   splice error   
289    Trace  
    A610G, C2650T   Heterozygous   M204V, Q884X   
    ins G3226   Heterozygous   1076 FsX   
293    Reduced  
    del 1822-1833   Homozygous   del V608-A611   
336    Absent  
    G175A   Homozygous   A59T   
    del 441A
 
Homozygous
 
147 Fs
 

 

UPN, mutations

Mutation type

Predicted effect

Munc13-4 expression
180    Absent  
    G175A   Heterozygous   A59T   
    753 + 1G > T   Homozygous   splice error   
277    Trace  
    del 3082C   Heterozygous   1028 Fs   
    G1241T, C2782T   Heterozygous   R414L, R928C   
249    Trace  
    A1847G   Homozygous   E616G   
237    Trace  
    A1847G   Heterozygous   E616G   
    G1241T, C2782T   Heterozygous   R414L, R928C   
225    Trace  
    A1847G   Heterozygous   E616G   
    753 + 1G > T   Heterozygous   splice error   
289    Trace  
    A610G, C2650T   Heterozygous   M204V, Q884X   
    ins G3226   Heterozygous   1076 FsX   
293    Reduced  
    del 1822-1833   Homozygous   del V608-A611   
336    Absent  
    G175A   Homozygous   A59T   
    del 441A
 
Homozygous
 
147 Fs
 

 

MUNC13-4 gene mutations in both alleles are listed in the table, including 8 patients with FHL. Munc 13-4 expression was tested by Western blot.

UPN indicates unique patient number; X, stop; Fs, frameshift.

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