Progressive muscular dystrophy in α-sarcoglycan–deficient mice

F Duclos, V Straub, SA Moore, DP Venzke… - The Journal of cell …, 1998 - rupress.org
Limb-girdle muscular dystrophy type 2D (LGMD 2D) is an autosomal recessive disorder
caused by mutations in the α-sarcoglycan gene. To determine how α-sarcoglycan deficiency …

Loss of the sarcoglycan complex and sarcospan leads to muscular dystrophy in β-sarcoglycan-deficient mice

K Araishi, T Sasaoka, M Imamura… - Human molecular …, 1999 - academic.oup.com
Abstract β-Sarcoglycan, one of the subunits of the sarcoglycan complex, is a
transmembranous glycoprotein which associates with dystrophin and is the molecule …

Disruption of the β-sarcoglycan gene reveals pathogenetic complexity of limb-girdle muscular dystrophy type 2E

M Durbeej, RD Cohn, RF Hrstka, SA Moore… - Molecular cell, 2000 - cell.com
Limb-girdle muscular dystrophy type 2E (LGMD 2E) is caused by mutations in the β-
sarcoglycan gene, which is expressed in skeletal, cardiac, and smooth muscle. β …

Functional rescue of the sarcoglycan complex in the BIO 14.6 hamster using δ-sarcoglycan gene transfer

KH Holt, LE Lim, V Straub, DP Venzke, F Duclos… - Molecular Cell, 1998 - cell.com
Four types of limb-girdle muscular dystrophy (LGMD) are known to be caused by mutations
in distinct sarcoglycan genes. The BIO 14.6 hamster is a model for sarcoglycan-deficient …

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 …

Sarcoglycan isoforms in skeletal muscle

LA Liu, E Engvall - Journal of Biological Chemistry, 1999 - ASBMB
The heterotetrameric sarcoglycan complex, composed of α-, β-, γ-, and δ-sarcoglycans, is an
important component of the dystrophin-associated glycoprotein assembly in striated muscle …

ζ-sarcoglycan, a novel component of the sarcoglycan complex, is reduced in muscular dystrophy

MT Wheeler, S Zarnegar… - Human molecular …, 2002 - academic.oup.com
The dystrophin glycoprotein complex (DGC) is found at the plasma membrane of muscle
cells, where it provides a link between the cytoskeleton and the extracellular matrix. A …

Characterization of δ-sarcoglycan, a novel component of the oligomeric sarcoglycan complex involved in limb-girdle muscular dystrophy

D Jung, F Duclos, B Apostol, V Straub, JC Lee… - Journal of Biological …, 1996 - ASBMB
The sarcoglycan complex is known to be involved in limb-girdle muscular dystrophy (LGMD)
and is composed of at least three proteins: α-, β-, and γ-sarcoglycan. δ-Sarcoglycan has now …

Muscle degeneration without mechanical injury in sarcoglycan deficiency

AA Hack, L Cordier, DI Shoturma… - Proceedings of the …, 1999 - National Acad Sciences
In humans, mutations in the genes encoding components of the dystrophin–glycoprotein
complex cause muscular dystrophy. Specifically, primary mutations in the genes encoding α …

ε-Sarcoglycan replaces α-sarcoglycan in smooth muscle to form a unique dystrophin-glycoprotein complex

V Straub, AJ Ettinger, M Durbeej, DP Venzke… - Journal of Biological …, 1999 - ASBMB
The sarcoglycan complex has been well characterized in striated muscle, and defects in its
components are associated with muscular dystrophy and cardiomyopathy. Here, we have …