No signal was detected in LAMP-2-deficient (LAMP-2-/y) brains.
(a) Immunoblot of hippocampal lysates from LAMP-2-deficient (LAMP-2-/y) mice and their wild-type (WT) littermates.
Representative histological brain sections from LAMP-2-deficient (LAMP-2-/y) mice and their wild-type (WT) littermates.
(a) LAMP-2-deficient (LAMP-2-/y) mice showed similar grip strength when compared to wild-type controls.
(a) Representative histological sections from LAMP-2-deficient (LAMP-2-/y) mice and their wild-type (WT) littermates stained with Periodic-Acid-Schiff (PAS).
Similarities in the pathological presentation of Danon disease in cardiac and skeletal muscle of humans and LAMP-2-deficient (LAMP-2-/y) mice [ 4, 28] that were of a mixed genetic background (SVJ-129/C57BL6-J (Harlan)) highlighthehe validity of this mouse as a model for the human disease.
(a) Immunoblot of lysates from brain lysates from wild-type (WT) and LAMP-2-deficient (LAMP-2-/y) mice as well as HeLa cells overexpressing eGFP, murine LAMP-1 (L1), murine LAMP-2A (L2A), murine LAMP-2B (L2B) or murine LAMP-2C (L2C).
It has been shown that unesterified cholesterol accumulates in endo/lysosomal compartments in LAMP double deficient cells [ 16].
Additional file 1: Neuropathology of LAMP-2-deficient mice.
LAMP-2-deficient mice were described previously [ 4, 30].
Filipin staining displayed storage of free cholesterol exclusively in the subiculum of LAMP-2-deficient mice.
