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Novel SPEG variants in a neonate with severe dilated cardiomyopathy and relatively mild hypotonia.

Hana Milena FujimotoMasanori FujimotoTakahiro SugiuraShigeharu NakaneYasuhiro WakanoEmi SatoHironori OshitaYasuko TogawaMari SugimotoTakenori KatoKazushi YasudaKanji MuramatsuShinji Saitoh
Published in: Human genome variation (2023)
Striated muscle preferentially expressed protein kinase (SPEG) variants have been reported to cause centronuclear myopathy associated with cardiac diseases. The severity of skeletal muscle symptoms and cardiac symptoms are presumably related to the location of the variant. Here, we report novel SPEG compound heterozygous pathological variants in a neonate with severe dilated cardiomyopathy and relatively mild hypotonia. This report expands the genotype-phenotype correlations of patients with SPEG variants.
Keyphrases
  • copy number
  • skeletal muscle
  • early onset
  • left ventricular
  • protein kinase
  • late onset
  • insulin resistance
  • type diabetes
  • heart failure
  • metabolic syndrome
  • physical activity
  • adipose tissue
  • duchenne muscular dystrophy