ceroid/neoplasms

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Role of palmitoyl-protein thioesterase in cell death: implications for infantile neuronal ceroid lipofuscinosis.

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Infantile neuronal ceroid lipofuscinosis (INCL) is a childhood neurodegenerative disease caused by the selective death of cortical and retinal neurons as the result of an inherited palmitoyl-protein thioesterase 1 (PPT1) deficiency. Neuronal death is common to many lysosomal storage diseases but it

Lysosomal serine protease CLN2 regulates tumor necrosis factor-alpha-mediated apoptosis in a Bid-dependent manner.

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Apoptosis is a highly organized, energy-dependent program by which multicellular organisms eliminate damaged, superfluous, and potentially harmful cells. Although caspases are the most prominent group of proteases involved in the apoptotic process, the role of lysosomes has only recently been

Lipomatous pleomorphic adenoma of the ceruminous gland.

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A case of lipomatous pleomorphic adenoma in the ceruminous gland is reported. A 69-year-old Japanese woman presented with a mass in the posterior wall of the cartilaginous external auditory canal. Light microscopic examination revealed a well-circumscribed tumor composed of tubular structures with

Brain-stem auditory evoked potentials in children with brain-stem or cerebellar dysfunction.

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Brain-stem auditory evoked potentials (BAEPs) were recorded in 23 children who had signs of brain-stem or cerebellar dysfunction. In patients with brain-stem gliomas, BAEPs were abnormal in all except one, in whom involvement of the brain-stem auditory pathway was limited to the midbrain tectum. The

Membranous fat necrosis in appendices epiploicae. A clinicopathological study.

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Membranous fat necrosis (MFN) is a degenerative process involving mature systemic adipose tissue. It is characterised by the presence of membranocystic foci surrounded by a lipophagic fibro-inflammatory reaction typical of fat necrosis. Membranocystic foci are cysts lined by an eosinophilic membrane

New functional aspects of cathepsin D and cathepsin E.

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Cathepsin D (CD) and cathepsin E are representative lysosomal and nonlysosomal aspartic proteinases, respectively, and play an important role in the degradation of proteins, the generation of bioactive proteins, antigen processing, etc. Recenty, several lines of evidence have suggested the

Pleiotropic effects of cathepsin D.

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Over the past decades, the paradigm that lysosomal enzymes participate only in non-specific protein degradation during cell death has changed. Studies conducted both in cell cultures and in animals defined the role of these enzymes that includes cathepsin D (CD). Knockout mice revealed the role of

Parasites and pathological conditions in the edible oyster, Crassostrea madrasensis (Preston), from the east and west coasts of India.

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A detailed pathological survey was carried out on the commercially important edible oyster, Crassostrea madrasensis (Preston), from two distinct coastal/brackish water ecosystems of south India. Samples were collected twice a year during wet and dry seasons from 2009 to 2012. Bacterial colonies in

NTP Toxicology and Carcinogenesis Studies of Tricresyl Phosphate (CAS No. 1330-78-5) in F344/N Rats and B6C3F1 Mice (Gavage and Feed Studies).

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Tricresyl phosphate is an organophosphate plasticizer widely used in vinyl plastics and as a fire retardant additive for hydraulic fluids. Toxicology and carcinogenesis studies were conducted by administering a mixed isomer preparation of 79% tricresyl phosphate esters (consisting of 21% tri-

Cathepsin D--many functions of one aspartic protease.

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For years, it has been held that cathepsin D (CD) is involved in rather non-specific protein degradation in a strongly acidic milieu of lysosomes. Studies with CD knock-out mice revealed that CD is not necessary for embryonal development, but it is indispensable for postnatal tissue homeostasis.

Crohn's-like acute severe colitis associated with Hermansky-Pudlak syndrome: A case report.

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Hermansky-Pudlak syndrome (HPS) is a rare autosomal recessive disorder characterized by oculocutaneous albinism, platelet storage pool deficiency and systemic complications associated with ceroid deposition in the reticuloendothelial system. HPS types 1 and 4 are associated with

Drosophila melanogaster as a model organism of brain diseases.

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Drosophila melanogaster has been utilized to model human brain diseases. In most of these invertebrate transgenic models, some aspects of human disease are reproduced. Although investigation of rodent models has been of significant impact, invertebrate models offer a wide variety of experimental

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