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Titolo:
Skeletal muscle Na currents in mice heterozygous for Six5 deficiency
Autore:
Mistry, DJ; Moorman, JR; Reddy, S; Mounsey, JP;
Indirizzi:
Univ Virginia, Hlth Sci Ctr, Dept Internal Med, Div Cardiovasc, Charlottesville, VA 22908 USA Univ Virginia Charlottesville VA USA 22908 Charlottesville, VA 22908 USA Univ Virginia, Hlth Sci Ctr, Dept Mol Physiol & Biol Phys, Charlottesville, VA 22908 USA Univ Virginia Charlottesville VA USA 22908 Charlottesville, VA 22908 USA Univ Virginia, Hlth Sci Ctr, Cardiovasc Res Ctr, Charlottesville, VA 22908USA Univ Virginia Charlottesville VA USA 22908 , Charlottesville, VA 22908USA Univ So Calif, Sch Med, Inst Med Genet, Los Angeles, CA 90033 USA Univ So Calif Los Angeles CA USA 90033 d Genet, Los Angeles, CA 90033 USA
Titolo Testata:
PHYSIOLOGICAL GENOMICS
fascicolo: 3, volume: 6, anno: 2001,
pagine: 153 - 158
SICI:
1094-8341(200108)6:3<153:SMNCIM>2.0.ZU;2-P
Fonte:
ISI
Lingua:
ENG
Soggetto:
DYSTROPHY PROTEIN-KINASE; MYOTONIC MUSCULAR-DYSTROPHY; CTG REPEAT; DECREASED EXPRESSION; TRINUCLEOTIDE REPEAT; REDUCES EXPRESSION; SODIUM-CHANNELS; TRIPLET REPEAT; MESSENGER-RNA; CELL-SHAPE;
Keywords:
myotonic muscular dystrophy; myotonic dystrophy kinase; ion channels; protein kinases;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
42
Recensione:
Indirizzi per estratti:
Indirizzo: Mounsey, JP Univ Virginia, Hlth Sci Ctr, Dept Internal Med, Div Cardiovasc, Box 6012,MR4 Bldg, Charlottesville, VA 22908 USA Univ Virginia Box 6012,MR4 Bldg Charlottesville VA USA 22908 A
Citazione:
D.J. Mistry et al., "Skeletal muscle Na currents in mice heterozygous for Six5 deficiency", PHYSIOL GEN, 6(3), 2001, pp. 153-158

Abstract

Myotonic dystrophy results from a trinucleotide repeat expansion between the myotonic dystrophy protein kinase gene (Dmpk), which encodes a serine-threonine protein kinase, and the Six5 gene, which encodes a homeodomain protein. The disease is characterized by late bursts of skeletal muscle Na channel openings, and this is recapitulated in Dmpk -/- and Dmpk +/- murine skeletal muscle. To test whether deficiency of the nearby Six5 gene also affected Na channel gating in murine skeletal muscle, we measured Na currents from cell-attached patches in Six5 +/- mice and age-matched wild-type and Dmpk +/- mice. Late bursts of Na channel activity were defined as an opening probability >10% measured from 10 to 110 ms after depolarization. There was no significant difference in the occurrence of late Na channel bursts in wild-type and Six5 +/- muscle, whereas in Dmpk +/- muscle there was greater than fivefold increase in late bursts (P< 0.001). Compared with wild-type mice, Na current amplitude was unchanged in Six5 +/- muscle, whereas in Dmpk /- muscle it was 36% reduced (P< 0.05). Thus, since Six5 +/- mice do not exhibit the Na channel gating abnormality of Dmpk deficiency, we conclude that Six5 deficiency does not contribute to the Na channel gating abnormalityseen in dystrophia myotonica patients.

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Documento generato il 26/01/21 alle ore 04:39:41