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Mutations of the slow muscle alpha-tropomyosin gene, TPM3, are a rare cause of nemaline myopathy.
Wattanasirichaigoon, D; Swoboda, K J; Takada, F; Tong, H-Q; Lip, V; Iannaccone, S T; Wallgren-Pettersson, C; Laing, N G; Beggs, A H.
Afiliación
  • Wattanasirichaigoon D; Genetics Division, Children's Hospital, Harvard Medical School, Boston, MA 02115, USA.
Neurology ; 59(4): 613-7, 2002 Aug 27.
Article en En | MEDLINE | ID: mdl-12196661
The alpha-tropomyosin-3 (TPM3) gene was screened in 40 unrelated patients with nemaline myopathy (NM). A single compound heterozygous patient was identified carrying one mutation that converts the stop codon to a serine and a second splicing mutation that is predicted to prevent inclusion of skeletal muscle exon IX. TPM3 mutations are a rare cause of NM, probably accounting for less than 3% of cases. The severity of cases with TPM3 mutations may vary from severe infantile to late childhood onset, slowly progressive forms.
Asunto(s)
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Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Tropomiosina / Miopatías Nemalínicas / Fibras Musculares de Contracción Lenta Tipo de estudio: Prognostic_studies Límite: Child / Child, preschool / Humans / Male Idioma: En Revista: Neurology Año: 2002 Tipo del documento: Article País de afiliación: Estados Unidos Pais de publicación: Estados Unidos
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Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Tropomiosina / Miopatías Nemalínicas / Fibras Musculares de Contracción Lenta Tipo de estudio: Prognostic_studies Límite: Child / Child, preschool / Humans / Male Idioma: En Revista: Neurology Año: 2002 Tipo del documento: Article País de afiliación: Estados Unidos Pais de publicación: Estados Unidos