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. 2011 Feb 22;2(1):33–42. doi: 10.1007/s12687-011-0038-0

Table 1.

Summary of positive diagnoses

Diagnosis No. affected per family Transmission Gene Nucleotide change Peptide change
Limb girdle muscular dystrophy, type 2D 3 AR SGCA Novel, homozygous 574C > T R192X
Limb girdle muscular dystrophy, type 2B 1 AR DYSF 2 novel heterozygous mutations, 2643 + 1G > A, 4018G > C Splice site, G1340R
Ataxia telangiectasia 2 AR ATM Novel Nt7985T > A V2662D
Duchenne muscular dystrophy 2 X-linked DMD Novel 3784delG 1262E > Frameshift KX20
Duchenne muscular dystrophy 2 X-linked DMD Novel 1423G > T 475E > X
Lafora disease 2 AR EPM2B Novel homozygous 560A > C H187P
Myotonia congenita 1 AR CLCN1 Homozygous 501C > G, heterozygous 313C > T, heterozygous 1672C > T F167L, R105C, P558S
Spinocerebellar ataxia, type 3 2 AD ATXN3 Heterozygous CAG repeat expansion (73, 14) Polyglutamine expansion
Spinocerebellar ataxia, type 7 3 AD ATXN7 Heterozygous CAG repeat expansion (56, 12) Polyglutamine expansion
Spinocerebellar ataxia, type 7 2 AD ATXN7 Heterozygous CAG repeat expansion (59, 11) Polyglutamine expansion
Kearns–Sayre syndrome 1 Mitoch Mitoch DNA 5,585 bp deletion N/A

AD autosomal dominant, AR autosomal recessive, Mitoch mitochondrial