about
Periplakin interactions with lens intermediate and beaded filamentsMyocardial fibrosis in desmin-related hypertrophic cardiomyopathy.Differential proteomic analysis of abnormal intramyoplasmic aggregates in desminopathyA novel desmin mutation leading to autosomal recessive limb-girdle muscular dystrophy: distinct histopathological outcomes compared with desminopathies.Mutation in BAG3 causes severe dominant childhood muscular dystrophy.Unusual multisystemic involvement and a novel BAG3 mutation revealed by NGS screening in a large cohort of myofibrillar myopathies.The desmin coil 1B mutation K190A impairs nebulin Z-disc assembly and destabilizes actin thin filaments.Desminopathies: pathology and mechanisms.Desmin mediates TNF-alpha-induced aggregate formation and intercalated disk reorganization in heart failure.Biomechanical characterization of myofibrillar myopathies.Early signs of architectural and biomechanical failure in isolated myofibers and immortalized myoblasts from desmin-mutant knock-in miceTruncation of alphaB-crystallin by the myopathy-causing Q151X mutation significantly destabilizes the protein leading to aggregate formation in transfected cells.Intermediate filaments and cellular mechanics.
P2860
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P2860
description
2007 nî lūn-bûn
@nan
2007年の論文
@ja
2007年論文
@yue
2007年論文
@zh-hant
2007年論文
@zh-hk
2007年論文
@zh-mo
2007年論文
@zh-tw
2007年论文
@wuu
2007年论文
@zh
2007年论文
@zh-cn
name
Primary desminopathies
@en
type
label
Primary desminopathies
@en
prefLabel
Primary desminopathies
@en
P2093
P2860
P921
P1476
Primary desminopathies
@en
P2093
Alexandra Vrabie
Hans H Goebel
Rolf Schröder
P2860
P304
P356
10.1111/J.1582-4934.2007.00057.X
P577
2007-05-01T00:00:00Z