Molecular dissection of the interaction of desmin with the C-terminal region of nebulin.
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Nebulin-deficient mice exhibit shorter thin filament lengths and reduced contractile function in skeletal muscleThe Nebulin family: an actin support groupThe nebulin SH3 domain is dispensable for normal skeletal muscle structure but is required for effective active load bearing in mouse.Bin1 SRC homology 3 domain acts as a scaffold for myofiber sarcomere assemblyNebulin binding impedes mutant desmin filament assembly.Nebulette is a powerful cytolinker organizing desmin and actin in mouse heartsSevere infantile-onset cardiomyopathy associated with a homozygous deletion in desmin.Nebulin plays a direct role in promoting strong actin-myosin interactions.Reduced myofibrillar connectivity and increased Z-disk width in nebulin-deficient skeletal muscle.Nebulin regulates actin filament lengths by a stabilization mechanism.New insights into the structural roles of nebulin in skeletal muscle.Nebulin, a major player in muscle health and diseaseMuscle giants: molecular scaffolds in sarcomerogenesis.Mutation update: the spectra of nebulin variants and associated myopathies.Cytoplasmic gamma-actin and tropomodulin isoforms link to the sarcoplasmic reticulum in skeletal muscle fibers.Increased desmin expression in hindlimb muscles of aging ratsThe desmin coil 1B mutation K190A impairs nebulin Z-disc assembly and destabilizes actin thin filaments.The sarcomeric protein nebulin: another multifunctional giant in charge of muscle strength optimizationNebulette knockout mice have normal cardiac function, but show Z-line widening and up-regulation of cardiac stress markers.Desminopathies: pathology and mechanisms.Nebulin interacts with CapZ and regulates thin filament architecture within the Z-discA myopathy-linked desmin mutation perturbs striated muscle actin filament architecture.Reduced thin filament length in nebulin-knockout skeletal muscle alters isometric contractile propertiesIntermediate filament diseases: desminopathy.Role of the sarcomeric Z-disc in the pathogenesis of cardiomyopathy.Lifting the nebula: novel insights into skeletal muscle contractility.Structure of giant muscle proteins.The sarcoplasmic reticulum: Actin and tropomodulin hit the links.Disorder profile of nebulin encodes a vernierlike position sensor for the sliding thin and thick filaments of the skeletal muscle sarcomere.Isolation of nebulin from rabbit skeletal muscle and its interaction with actin.Type III Intermediate Filaments Desmin, Glial Fibrillary Acidic Protein (GFAP), Vimentin, and Peripherin.Actin-related myopathy without any missense mutation in the ACTA1 gene.Expression of multiple nebulin isoforms in human skeletal muscle and brain.Identification of the rare compound heterozygous variants in the NEB gene in a Korean family with intellectual disability, epilepsy and early-childhood-onset generalized muscle weakness.
P2860
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P2860
Molecular dissection of the interaction of desmin with the C-terminal region of nebulin.
description
2002 nî lūn-bûn
@nan
2002年の論文
@ja
2002年学术文章
@wuu
2002年学术文章
@zh-cn
2002年学术文章
@zh-hans
2002年学术文章
@zh-my
2002年学术文章
@zh-sg
2002年學術文章
@yue
2002年學術文章
@zh
2002年學術文章
@zh-hant
name
Molecular dissection of the interaction of desmin with the C-terminal region of nebulin.
@en
Molecular dissection of the interaction of desmin with the C-terminal region of nebulin.
@nl
type
label
Molecular dissection of the interaction of desmin with the C-terminal region of nebulin.
@en
Molecular dissection of the interaction of desmin with the C-terminal region of nebulin.
@nl
prefLabel
Molecular dissection of the interaction of desmin with the C-terminal region of nebulin.
@en
Molecular dissection of the interaction of desmin with the C-terminal region of nebulin.
@nl
P356
P1476
Molecular dissection of the interaction of desmin with the C-terminal region of nebulin.
@en
P2093
Carol Gregorio
Siegfried Labeit
P304
P356
10.1006/JSBI.2002.4457
P577
2002-01-01T00:00:00Z