A mutation in alpha-tropomyosin(slow) affects muscle strength, maturation and hypertrophy in a mouse model for nemaline myopathy.
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Nebulin regulates thin filament length, contractility, and Z-disk structure in vivo.Respiratory assessment in centronuclear myopathiesPathophysiological concepts in the congenital myopathies: blurring the boundaries, sharpening the focusNormal myofibrillar development followed by progressive sarcomeric disruption with actin accumulations in a mouse Cfl2 knockout demonstrates requirement of cofilin-2 for muscle maintenanceKLHL40 deficiency destabilizes thin filament proteins and promotes nemaline myopathyLeiomodin-3-deficient mice display nemaline myopathy with fast-myofiber atrophyCongenital myopathies: diseases of the actin cytoskeleton.Actin nemaline myopathy mouse reproduces disease, suggests other actin disease phenotypes and provides cautionary note on muscle transgene expression.Loss of Par-1a/MARK3/C-TAK1 kinase leads to reduced adiposity, resistance to hepatic steatosis, and defective gluconeogenesis.Alterations at the cross-bridge level are associated with a paradoxical gain of muscle function in vivo in a mouse model of nemaline myopathyMice lacking skeletal muscle actin show reduced muscle strength and growth deficits and die during the neonatal periodNebulin deficiency in adult muscle causes sarcomere defects and muscle-type-dependent changes in trophicity: novel insights in nemaline myopathyGene expression patterns underlying parasite-induced alterations in host behaviour and life history.Skeletal muscle repair in a mouse model of nemaline myopathy.Tropomyosin-based regulation of the actin cytoskeleton in time and space.Multimodal MRI and (31)P-MRS investigations of the ACTA1(Asp286Gly) mouse model of nemaline myopathy provide evidence of impaired in vivo muscle function, altered muscle structure and disturbed energy metabolismTropomyosin isoforms and reagentsIdentification of FHL1 as a regulator of skeletal muscle mass: implications for human myopathy.Rescue of skeletal muscle alpha-actin-null mice by cardiac (fetal) alpha-actin.Designing heart performance by gene transfer.Disease severity and thin filament regulation in M9R TPM3 nemaline myopathy.Ca2+ dysregulation in Ryr1(I4895T/wt) mice causes congenital myopathy with progressive formation of minicores, cores, and nemaline rodsSarcomere Dysfunction in Nemaline Myopathy.Distinct underlying mechanisms of limb and respiratory muscle fiber weaknesses in nemaline myopathy.Exome sequencing reveals a nebulin nonsense mutation in a dog model of nemaline myopathy.Chromatin structure analysis enables detection of DNA insertions into the mammalian nuclear genome.Misregulation of calcium-handling proteins promotes hyperactivation of calcineurin-NFAT signaling in skeletal muscle of DM1 mice.Molecular Genetics of Nemaline MyopathyHypertrophy and dietary tyrosine ameliorate the phenotypes of a mouse model of severe nemaline myopathy
P2860
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P2860
A mutation in alpha-tropomyosin(slow) affects muscle strength, maturation and hypertrophy in a mouse model for nemaline myopathy.
description
2001 nî lūn-bûn
@nan
2001 թուականի Փետրուարին հրատարակուած գիտական յօդուած
@hyw
2001 թվականի փետրվարին հրատարակված գիտական հոդված
@hy
2001年の論文
@ja
2001年論文
@yue
2001年論文
@zh-hant
2001年論文
@zh-hk
2001年論文
@zh-mo
2001年論文
@zh-tw
2001年论文
@wuu
name
A mutation in alpha-tropomyosi ...... e model for nemaline myopathy.
@ast
A mutation in alpha-tropomyosi ...... e model for nemaline myopathy.
@en
type
label
A mutation in alpha-tropomyosi ...... e model for nemaline myopathy.
@ast
A mutation in alpha-tropomyosi ...... e model for nemaline myopathy.
@en
prefLabel
A mutation in alpha-tropomyosi ...... e model for nemaline myopathy.
@ast
A mutation in alpha-tropomyosi ...... e model for nemaline myopathy.
@en
P2093
P356
P1476
A mutation in alpha-tropomyosi ...... e model for nemaline myopathy.
@en
P2093
Corbett MA
Dunglison GF
Gunning PW
Hardeman EC
Robinson CS
Stewart AW
P304
P356
10.1093/HMG/10.4.317
P577
2001-02-01T00:00:00Z