TY - JOUR
T1 - Functional diversity of dystroglycan
AU - Bozzi, Manuela
AU - Morlacchi, Simona
AU - Bigotti, Maria Giulia
AU - Sciandra, Francesca
AU - Brancaccio, Andrea
PY - 2009
Y1 - 2009
N2 - During the last 15 years, following its identification and first detailed molecular characterization, the
dystroglycan (DG) complex has taken centre stage in biology and biomedicine. Functions in different cells
and tissues have been identified for this complex, ranging from its typical role in skeletal muscle as a
sarcolemmal stabilizer, highlighted by the recently identified “secondary dystroglycanopathies”, to a variety
of very diverse functions including embryogenesis, cancer progression, virus particle entry and cell signalling.
Such functional promiscuity can be in part explained when considering the multiple domain organization of
the two DG subunits, the extracellular α-DG and the transmembrane β-DG, that has been largely scrutinized,
but only in part unraveled, exploiting a variety of recombinant and transgenic approaches. Herein, while
rapidly recapitulating some of the functions that nowadays can be assigned safely to each DG domain, we
also try to envisage a sort of worry list featuring and dwelling on some of the most compelling ”mysteries”
that should be solved to finally understand DG's functional diversity.
AB - During the last 15 years, following its identification and first detailed molecular characterization, the
dystroglycan (DG) complex has taken centre stage in biology and biomedicine. Functions in different cells
and tissues have been identified for this complex, ranging from its typical role in skeletal muscle as a
sarcolemmal stabilizer, highlighted by the recently identified “secondary dystroglycanopathies”, to a variety
of very diverse functions including embryogenesis, cancer progression, virus particle entry and cell signalling.
Such functional promiscuity can be in part explained when considering the multiple domain organization of
the two DG subunits, the extracellular α-DG and the transmembrane β-DG, that has been largely scrutinized,
but only in part unraveled, exploiting a variety of recombinant and transgenic approaches. Herein, while
rapidly recapitulating some of the functions that nowadays can be assigned safely to each DG domain, we
also try to envisage a sort of worry list featuring and dwelling on some of the most compelling ”mysteries”
that should be solved to finally understand DG's functional diversity.
KW - dystroglycan
KW - dystroglycan
UR - http://hdl.handle.net/10807/32760
M3 - Article
SN - 0945-053X
VL - 2009
SP - 179
EP - 187
JO - Matrix Biology
JF - Matrix Biology
ER -