Biomimetic models to investigate membrane biophysics affecting lipid–protein interaction

J Sarkis, V Vié - Frontiers in bioengineering and biotechnology, 2020 - frontiersin.org
Biological membranes are highly dynamic in their ability to orchestrate vital mechanisms
including cellular protection, organelle compartmentalization, cellular biomechanics, nutrient …

Elevated concentrations cause upright alpha-synuclein conformation at lipid interfaces

SJ Roeters, K Strunge, KB Pedersen… - Nature …, 2023 - nature.com
The amyloid aggregation of α-synuclein (α S), related to Parkinson's disease, can be
catalyzed by lipid membranes. Despite the importance of lipid surfaces, the 3D-structure and …

Dystrophin contains multiple independent membrane-binding domains

J Zhao, K Kodippili, Y Yue, CH Hakim… - Human molecular …, 2016 - academic.oup.com
Dystrophin is a large sub-sarcolemmal protein. Its absence leads to Duchenne muscular
dystrophy (DMD). Binding to the sarcolemma is essential for dystrophin to protect muscle …

Targeted RNA-Seq profiling of splicing pattern in the DMD gene: exons are mostly constitutively spliced in human skeletal muscle

AL Bougé, E Murauer, E Beyne, J Miro, J Varilh… - Scientific reports, 2017 - nature.com
We have analysed the splicing pattern of the human Duchenne Muscular Dystrophy (DMD)
transcript in normal skeletal muscle. To achieve depth of coverage required for the analysis …

Increased nonHDL cholesterol levels cause muscle wasting and ambulatory dysfunction in the mouse model of LGMD2B

SL Sellers, N Milad, Z White, C Pascoe, R Chan… - Journal of Lipid …, 2018 - ASBMB
Progressive limb and girdle muscle atrophy leading to loss of ambulation is a hallmark of
dysferlinopathies, which include limb-girdle muscular dystrophy type 2B and Miyoshi …

The role of langmuir monolayers to understand biological events

L Caseli, TM Nobre, AP Ramos… - Recent Progress in …, 2015 - ACS Publications
In this chapter, we present an overview about different roles of Langmuir monolayers as
biomembrane models, contributing to understand biological phenomena or help develop …

Diverse action of selected statins on skeletal muscle cells—an attempt to explain the protective effect of geranylgeraniol (GGOH) in statin-associated myopathy (SAM)

A Jaśkiewicz, B Pająk, M Łabieniec-Watała… - Journal of Clinical …, 2019 - mdpi.com
The present study is centered on molecular mechanisms of the cytoprotective effect of
geranylgeraniol (GGOH) in skeletal muscle harmed by statin-associated myopathy (SAM) …

The Donnan-dominated resting state of skeletal muscle fibers contributes to resilience and longevity in dystrophic fibers

CE Morris, JJ Wheeler, B Joos - Journal of General Physiology, 2021 - rupress.org
Duchenne muscular dystrophy (DMD) is an X-linked dystrophin-minus muscle-wasting
disease. Ion homeostasis in skeletal muscle fibers underperforms as DMD progresses. But …

Fluorescence study of the effect of cholesterol on spectrin–aminophospholipid interactions

M Mitra, M Patra, A Chakrabarti - European Biophysics Journal, 2015 - Springer
The ability of the membrane skeletal protein spectrin to interact with phospholipids, and
aminophospholipids in particular, in both natural and model membranes, is well …

Dystrophin hot-spot mutants leading to Becker muscular dystrophy insert more deeply into membrane models than the native protein

S Ameziane-Le Hir, G Paboeuf, C Tascon… - Biochemistry, 2016 - ACS Publications
Dystrophin (DYS) is a membrane skeleton protein whose mutations lead to lethal Duchenne
muscular dystrophy or to the milder Becker muscular dystrophy (BMD). One third of BMD “in …