Calpain 3 deficiency is associated with myonuclear apoptosis and profound perturbation of the IkappaB alpha/NF-kappaB pathway in limb-girdle muscular dystrophy type 2A.
Baghdiguian
S
S
Laboratoire de Dynamique Moléculaire des Interactions Membranaires, CNRS-UMR 5539, Université Montpellier II, France.
Martin
M
M
Richard
I
I
Pons
F
F
Astier
C
C
Bourg
N
N
Hay
R T
RT
Chemaly
R
R
Halaby
G
G
Loiselet
J
J
Anderson
L V
LV
Lopez de Munain
A
A
Fardeau
M
M
Mangeat
P
P
Beckmann
J S
JS
Lefranc
G
G
D000293:Adolescent; D000328:Adult; D017209:Apoptosis; D001692:Biological Transport; D002154:Calpain / Q000172:deficiency* / Q000378:metabolism; D002451:Cell Compartmentation; D002467:Cell Nucleus / Q000473:pathology*; D002648:Child; D004268:DNA-Binding Proteins / Q000235:genetics / Q000378:metabolism*; D005260:Female; D005333:Fetus; D005455:Fluorescent Antibody Technique; D006801:Humans; D020672:I-kappa B Proteins; D008297:Male; D018613:Microscopy, Confocal; D018482:Muscle, Skeletal / Q000378:metabolism / Q000473:pathology; D009136:Muscular Dystrophies / Q000145:classification / Q000201:enzymology / Q000378:metabolism* / Q000473:pathology; D000072000:NF-KappaB Inhibitor alpha; D016328:NF-kappa B / Q000037:antagonists & inhibitors / Q000378:metabolism*; D011994:Recombinant Proteins / Q000378:metabolism
D004268:DNA-Binding Proteins; D020672:I-kappa B Proteins; D016328:NF-kappa B; C000606204:NFKBIA protein, human; D011994:Recombinant Proteins; D000072000:NF-KappaB Inhibitor alpha; D002154:Calpain