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Three novel SLC37A4 variants in glycogen storage disease type 1b and a literature review.
Wang, Zhuolin; Zhao, Ruiqin; Jia, Xiaoyun; Li, Xiaolei; Ma, Li; Fu, Haiyan.
Afiliación
  • Wang Z; Department of Gastroenterology, Hebei Children's Hospital, 133 Jianhua South Street, Shijiazhuang 050031, Hebei Province, China.
  • Zhao R; Department of Gastroenterology, Hebei Children's Hospital, 133 Jianhua South Street, Shijiazhuang 050031, Hebei Province, China.
  • Jia X; Department of Gastroenterology, Hebei Children's Hospital, 133 Jianhua South Street, Shijiazhuang 050031, Hebei Province, China.
  • Li X; Department of Gastroenterology, Hebei Children's Hospital, 133 Jianhua South Street, Shijiazhuang 050031, Hebei Province, China.
  • Ma L; Department of Neonatology, Hebei Children's Hospital, 133 Jianhua South Street, Shijiazhuang 050031, Hebei Province, China.
  • Fu H; Department of Gastroenterology, Hebei Children's Hospital, 133 Jianhua South Street, Shijiazhuang 050031, Hebei Province, China.
J Int Med Res ; 51(12): 3000605231216633, 2023 Dec.
Article en En | MEDLINE | ID: mdl-38087503
Glycogen storage disease type 1b (GSD1b) is a rare genetic disorder, resulting from mutations in the SLC37A4 gene located on chromosome 11q23.3. Although the SLC37A4 gene has been identified as the pathogenic gene for GSD1b, the complete variant spectrum of this gene remains to be fully elucidated. In this study, we present three patients diagnosed with GSD1b through genetic testing. We detected five variants of the SLC37A4 gene in these three patients, with three of these mutations (p. L382Pfs*15, p. G117fs*28, and p. T312Sfs*13) being novel variants not previously reported in the literature. We also present a literature review and general overview of the currently reported SLC37A4 gene variants. Our study expands the mutation spectrum of SLC37A4, which may help enable genetic testing to facilitate prompt diagnosis, appropriate intervention, and genetic counseling for affected families.
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Texto completo: 1 Colección: 01-internacional Banco de datos: MEDLINE Asunto principal: Enfermedad del Almacenamiento de Glucógeno / Enfermedad del Almacenamiento de Glucógeno Tipo I Límite: Humans Idioma: En Revista: J Int Med Res Año: 2023 Tipo del documento: Article País de afiliación: China

Texto completo: 1 Colección: 01-internacional Banco de datos: MEDLINE Asunto principal: Enfermedad del Almacenamiento de Glucógeno / Enfermedad del Almacenamiento de Glucógeno Tipo I Límite: Humans Idioma: En Revista: J Int Med Res Año: 2023 Tipo del documento: Article País de afiliación: China