Dystroglycan: an extracellular matrix receptor linked to the cytoskeleton

MD Henry, KP Campbell - Current opinion in cell biology, 1996 - Elsevier
Dystroglycan provides a crucial linkage between the cytoskeleton and the basement
membrane for skeletal muscle cells. Disruption of this linkage leads to various forms of …

From dystrophinopathy to sarcoglycanopathy: evolution of a concept of muscular dystrophy

E Ozawa, S Noguchi, Y Mizuno… - Muscle & Nerve …, 1998 - Wiley Online Library
Duchenne and Becker muscular dystrophies are collectively termed dystrophinopathy.
Dystrophinopathy and severe childhood autosomal recessive muscular dystrophy …

Forced expression of dystrophin deletion constructs reveals structure-function correlations.

JA Rafael, GA Cox, K Corrado, D Jung… - The Journal of cell …, 1996 - rupress.org
Dystrophin plays an important role in skeletal muscle by linking the cytoskeleton and the
extracellular matrix. The amino terminus of dystrophin binds to actin and possibly other …

Membrane targeting and stabilization of sarcospan is mediated by the sarcoglycan subcomplex

RH Crosbie, CS Lebakken, KH Holt… - The Journal of cell …, 1999 - rupress.org
The dystrophin–glycoprotein complex (DGC) is a multisubunit complex that spans the
muscle plasma membrane and forms a link between the F-actin cytoskeleton and the …

The limb-girdle muscular dystrophies—multiple genes, multiple mechanisms

KMD Bushby - Human molecular genetics, 1999 - academic.oup.com
In the field of muscular dystrophy, advances in understanding the molecular basis of the
various disorders in this group have been rapidly translated into readily applicable …

ε-sarcoglycan, a broadly expressed homologue of the gene mutated in limb-girdle muscular dystrophy 2D

AJ Ettinger, G Feng, JR Sanes - Journal of Biological Chemistry, 1997 - ASBMB
The sarcoglycans are transmembrane components of the dystrophin-glycoprotein complex,
which links the cytoskeleton to the extracellular matrix in adult muscle fibers. Mutations in all …

Assembly of the sarcoglycan complex: insights for muscular dystrophy

KH Holt, KP Campbell - Journal of Biological Chemistry, 1998 - ASBMB
Four unique transmembrane glycoproteins comprise the sarcoglycan complex in striated
muscle. The sarcoglycan complex contributes to maintenance of sarcolemma integrity. A …

A founder mutation in the γ-sarcoglycan gene of Gypsies possibly predating their migration out of India

F Piccolo, M Jeanpierre, F Leturcq… - Human molecular …, 1996 - academic.oup.com
We investigated the molecular basis of a severe form of early onset autosomal recessive
muscular dystrophy with sarcoglycan (SG) deficiency in seven large Gypsy families living in …

Molecular organization of sarcoglycan complex in mouse myotubes in culture

Y Chan, CG Bönnemann, HGW Lidov… - The Journal of cell …, 1998 - rupress.org
The sarcoglycans are a complex of four transmembrane proteins (α, β, γ, and δ) which are
primarily expressed in skeletal muscle and are closely associated with dystrophin and the …

The sarcoglycan complex in limb–girdle muscular dystrophy

LE Lim, KP Campbell - Current opinion in neurology, 1998 - journals.lww.com
The involvement of the sarcoglycan complex in the pathogenesis of muscular dystrophy is
becoming increasingly clear. Sarcoglycan gene mutations lead to four forms of autosomal …