On noxious desmin: functional effects of a novel heterozygous desmin insertion mutation on the extrasarcomeric desmin cytoskeleton and mitochondria.
about
From bioblasts to mitochondria: ever expanding roles of mitochondria in cell physiologyIntermediate Filaments as Organizers of Cellular Space: How They Affect Mitochondrial Structure and FunctionThe specificity of the interaction between αB-crystallin and desmin filaments and its impact on filament aggregation and cell viability.A mutation in the dimerization domain of filamin c causes a novel type of autosomal dominant myofibrillar myopathy.A novel desmin mutation leading to autosomal recessive limb-girdle muscular dystrophy: distinct histopathological outcomes compared with desminopathies.Compound heterozygosity of predicted loss-of-function DES variants in a family with recessive desminopathyDesmin-related cardiomyopathy: an unfolding storyDesminopathies in muscle disease.Voltage-Dependent Anion Channel 1(VDAC1) Participates the Apoptosis of the Mitochondrial Dysfunction in Desminopathy.Desminopathies: pathology and mechanisms.Desmin mediates TNF-alpha-induced aggregate formation and intercalated disk reorganization in heart failure.Primary desminopathiesDistinct muscle imaging patterns in myofibrillar myopathies.Tragedy in a heartbeat: malfunctioning desmin causes skeletal and cardiac muscle diseaseMitochondrial dysfunction in myofibrillar myopathy.Intermediate filament diseases: desminopathy.Valosin-containing protein disease: inclusion body myopathy with Paget's disease of the bone and fronto-temporal dementia.Mitochondrial abnormalities in the myofibrillar myopathies.Early signs of architectural and biomechanical failure in isolated myofibers and immortalized myoblasts from desmin-mutant knock-in miceMutant desmin substantially perturbs mitochondrial morphology, function and maintenance in skeletal muscle tissueAbnormal expression of RNA polymerase II-associated proteins in muscle of patients with myofibrillar myopathies.Disruption of desmin-mitochondrial architecture in patients with regurgitant mitral valves and preserved ventricular function.Desmin common mutation is associated with multi-systemic disease manifestations and depletion of mitochondria and mitochondrial DNAHsp27-2D-gel electrophoresis is a diagnostic tool to differentiate primary desminopathies from myofibrillar myopathies.The role of heat shock proteins and co-chaperones in heart failure.Molecular insights into cardiomyopathies associated with desmin (DES) mutations
P2860
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P2860
On noxious desmin: functional effects of a novel heterozygous desmin insertion mutation on the extrasarcomeric desmin cytoskeleton and mitochondria.
description
2003 nî lūn-bûn
@nan
2003年の論文
@ja
2003年論文
@yue
2003年論文
@zh-hant
2003年論文
@zh-hk
2003年論文
@zh-mo
2003年論文
@zh-tw
2003年论文
@wuu
2003年论文
@zh
2003年论文
@zh-cn
name
On noxious desmin: functional ...... cytoskeleton and mitochondria.
@en
type
label
On noxious desmin: functional ...... cytoskeleton and mitochondria.
@en
prefLabel
On noxious desmin: functional ...... cytoskeleton and mitochondria.
@en
P2093
P2860
P50
P356
P1476
On noxious desmin: functional ...... cytoskeleton and mitochondria
@en
P2093
Bertrand Goudeau
Christoph S Clemen
Dieter O Fürst
Dirk Fischer
Jens Reimann
Klaus Zerres
Monique Casteras Simon
Patrick Vicart
Peter F M van der Ven
Rolf Schröder
P2860
P304
P356
10.1093/HMG/DDG060
P577
2003-03-01T00:00:00Z