Infantile neuronal ceroid lipofuscinosis (INCL) is a severe lysosomal disease caused by mutations in the CLN1 gene, which encodes the soluble lysosomal enzyme Palmitoyl- Protein-Thioesterase-1 (PPT1) and result in osmiophilic
granules accumulating in lysosomes and leading to neuroinflammation, neurodegeneration and death.
The morphology of the fixed cells was observed, and round osmiophilic
structures were identified as lipid droplets, which were then counted under phase-contrast microscopy using the 100x objective lens in 50 consecutively scanned leukocytes in each coverslip.
Cytoplasm contained osmiophilic
lamellar bodies, mitochondria and Golgi body.
Ultrastructural analysis shows intracellular osmiophilic
, lamellated membrane structures with a concentric pattern called myelin bodies or with elongated stripes called zebra bodies.
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited cerebral small and medium vessel disease caused by mutations in NOTCH3 , which encodes Notch3 receptor expressed in vascular smooth muscle cells (VSMC).[sup], Pathological changes include deposition of granular osmiophilic
material on the surface of smooth muscle cells with subsequent fibrosis in the wall of arterioles.[sup] Clinical manifestations of CADASIL include migraine with aura, subcortical ischemic events, mood disturbances, apathy, and cognitive impairment.[sup] Brain magnetic resonance imaging (MRI) is used for diagnosis and follow-up in these patients.
Rare, small, subendothelial osmiophilic
electron-dense deposits were identified.
 independently portrayed a "lipidized" variant of ependymoma confirming the presence of variable sized osmiophilic
lipid droplets in the material submitted for ultrastructural examination.
Some mitochondrial matrices were dense with osmiophilic
particles, and the ridges were disordered and difficult to distinguish.
Small perforating cerebral arteries are most frequently affected, with an accumulation of granular and osmiophilic
substances within the vascular smooth muscle cell membrane (VSMC) as the basic pathological finding [19,20].
Moreover, starting from 8 h up to 24 h of CisPt treatment, autophagolysosomes appeared largely filled with heterogeneous materials and often associated with scattered round and dense mitochondria with amorphous cristae; finally, at 24 h, several clusters of autophagic vacuoles, osmiophilic
myelinic figures that migrated at the end of the fibers or beneath the sarcolemma, and apoptotic nuclei with condensed chromatin were detected (Figures 4(h)-4(i)).
7a), for example, it contained compact and smooth cell wall layers, typical elliptical chloroplasts containing 8-12 grana with large and plump osmiophilic
granules, and the mitochondria showed an elliptical shape in intracellular compartment (Fig.