Increased number of caveolae and caveolin-3 overexpression in Duchenne muscular dystrophy.
Caveolae are small pockets or invaginations localized at the plasma membrane. Caveolins are the principal protein components of caveolae and play an important structural role in the formation of caveolae membranes. Here, we studied by freeze fracture and immunological techniques the spatial organization of caveolae at the muscle cell plasma membrane and the expression of caveolin-3 in Duchenne muscular dystrophy (DMD) muscle fibers. In DMD muscle, we found an increased number of caveolae at the sarcolemma that corresponds to an overexpression of caveolin-3 by immunohistochemistry and by Western blot analysis. These findings suggest a possible role for caveolae and caveolin-3 in the pathogenesis of DMD.
Repetto
S
S
Istituto Gaslini, Università di Genova, Largo Gaslini 5, Genoa, 16147, Italy.
Bado
M
M
Broda
P
P
Lucania
G
G
Masetti
E
E
Sotgia
F
F
Carbone
I
I
Pavan
A
A
Bonilla
E
E
Cordone
G
G
Lisanti
M P
MP
Minetti
C
C
D000293:Adolescent; D015153:Blotting, Western; D051244:Caveolin 3; D022461:Caveolins; D002462:Cell Membrane / Q000737:chemistry* / Q000648:ultrastructure*; D002648:Child; D002675:Child, Preschool; D005614:Freeze Fracturing; D006801:Humans; D007150:Immunohistochemistry; D008565:Membrane Proteins / Q000032:analysis*; D008875:Middle Aged; D009124:Muscle Proteins / Q000032:analysis; D018482:Muscle, Skeletal / Q000737:chemistry / Q000648:ultrastructure*; D009136:Muscular Dystrophies / Q000378:metabolism / Q000473:pathology*; D012508:Sarcolemma / Q000648:ultrastructure
D051244:Caveolin 3; D022461:Caveolins; D008565:Membrane Proteins; D009124:Muscle Proteins