Cardioprotective Effect of Whole Body Periodic Acceleration in Dystrophic Phenotype mdx Rodent
Frontiers in Physiology, 2021 Duchenne muscular dystrophy (DMD) is characterized by progressive muscle wasting and the development of a dilated cardiomyopathy (DCM), which is the leading cause of death in DMD patients.
Arkady Uryash +5 more
doaj +1 more source
Identification, Characterization, and Localization of a Novel Kidney Polycystin-1-Polycystin-2 Complex [PDF]
, 2002 The functions of the two proteins defective in autosomal dominant polycystic kidney disease, polycystin-1 and polycystin-2, have not been fully clarified, but it has been hypothesized that they may heterodimerize to form a "polycystin complex" involved ...
Ackermann +43 more
core +1 more source
Characterization of Dystrophin and Utrophin Diversity in the Mouse [PDF]
Human Molecular Genetics, 1999 Utrophin is a 400 kDa autosomal homolog of dystrophin and a component of the submembranous cytoskeleton. While multiple dystrophin isoforms have been identified along with alternatively spliced products, to date only two different mRNA species of utrophin have been identified.
Jill A. Rafael +8 more
openaire +3 more sources
Drp2 and Periaxin Form Cajal Bands with Dystroglycan But Have Distinct Roles in Schwann Cell Growth [PDF]
, 2012 Cajal bands are cytoplasmic channels flanked by appositions where the abaxonal surface of Schwann cell myelin apposes and adheres to the overlying plasma membrane.
Brophy, Peter J. +4 more
core +1 more source
Actin assembly ruptures the nuclear envelope by prying the lamina away from nuclear pores and nuclear membranes in starfish oocytes. [PDF]
, 2020 The nucleus of oocytes (germinal vesicle) is unusually large and its nuclear envelope (NE) is densely packed with nuclear pore complexes (NPCs) stockpiled for embryonic development. We showed that breakdown of this specialized NE is mediated by an Arp2/3-
Avilov, I. +6 more
core +1 more source
Heregulin‐induced epigenetic regulation of the utrophin‐A promoter [PDF]
FEBS Letters, 2007 Utrophin is the autosomal homolog of dystrophin, the product of the Duchenne's muscular dystrophy (DMD) locus. Utrophin is of therapeutic interest since its over‐expression can compensate dystrophin's absence. Utrophin is enriched at neuromuscular junctions due to heregulin‐mediated utrophin‐A promoter activation.
Utpal Basu +8 more
openaire +5 more sources
WH2 and proline-rich domains of WASP-family proteins collaborate to accelerate actin filament elongation. [PDF]
, 2017 WASP-family proteins are known to promote assembly of branched actin networks by stimulating the filament-nucleating activity of the Arp2/3 complex.
Akin, Orkun +6 more
core +1 more source
PLoS ONE, 2016 Duchenne muscular dystrophy is a severe and currently incurable progressive neuromuscular condition, caused by mutations in the DMD gene that result in the inability to produce dystrophin. Lack of dystrophin leads to loss of muscle fibres and a reduction
Narinder Janghra +6 more
doaj +1 more source
Neurobiology of Disease, 1999 Duchenne muscular dystrophy (DMD), a severe X-linked recessive disorder which results in progressive muscle degeneration, is due to a lack of dystrophin, a membrane cytoskeletal protein.
Emmanuel Chaubourt +5 more
doaj +1 more source
Preventing phosphorylation of dystroglycan ameliorates the dystrophic phenotype in mdx mouse [PDF]
, 2012 Loss of dystrophin protein due to mutations in the DMD gene causes Duchenne muscular dystrophy. Dystrophin loss also leads to the loss of the dystrophin glycoprotein complex (DGC) from the sarcolemma which contributes to the dystrophic phenotype ...
Andrew Mitchell +55 more
core +2 more sources