acid-phosphatase and Central-Nervous-System-Diseases

Lysosomal acid phosphatase (LAP) is a tartrate-sensitive enzyme with ubiquitous expression. Neither the physiological substrates nor the functional significance is known. Mice with a deficiency of LAP generated by targeted disruption of the LAP gene are fertile and develop normally. Microscopic examination of various peripheral organs revealed progredient lysosomal storage in podocytes and tubular epithelial cells of the kidney, with regionally different ultrastructural appearance of the stored material. Within the central nervous system, lysosomal storage was detected to a regionally different extent in microglia, ependymal cells, and astroglia concomitant with the development of a progressive astrogliosis and microglial activation. Whereas behavioral and neuromotor analyses were unable to distinguish between control and deficient mice, approximately 7% of the deficient animals developed generalized seizures. From the age of 6 months onward, conspicuous alterations of bone structure became apparent, resulting in a kyphoscoliotic malformation of the lower thoracic vertebral column. We conclude from these findings that LAP has a unique function in only a subset of cells, where its deficiency causes the storage of a heterogeneously appearing material in lysosomes. The causal relationship of the enzyme defect to the clinical manifestations remains to be determined.

Topics: Acid Phosphatase; Animals; Antigens, CD; Bone and Bones; Cathepsin D; Central Nervous System Diseases; Fibroblasts; Kidney Diseases; Lysosomal Membrane Proteins; Lysosomal Storage Diseases; Lysosomes; Membrane Glycoproteins; Mice; Microglia; Phenotype; Seizures; Tartrates

The activities of acid phosphatase, hexosaminidase, beta-galactosidase, Mg2+-stimulated Na+K+ATPase, fumarase and ATP:citrate lyase were measured in grey matter of rabbit spinal cord 7-8 days after intra-ventricular or intra-cisternal injection of aluminium. RNA, DNA, and water content were measured in whole spinal cords. Choline acetyltransferase (CAT) and acetylcholinesterase were assayed in dorsal grey matter of the cord, which contained no aluminium-induced neurofilament accumulations (NFAs), and ventral grey matter, which had large numbers of such NFAs. CAT was also assayed in the hypoglossal nerve. None of these measures were consistently altered in the aluminium treated rabbits, although the activity of beta-galactosidase was increased in the NFA-free caudate nucleus of rabbits given aluminium intra-ventricularly, possibly due to the presence of phagocytes on the ventricular surface of the caudate. It is concluded that neither aluminium nor its induced NFAs has a gross effect on neuronal metabolism within 7-8 days.

Topics: Acid Phosphatase; Aluminum; Animals; ATP Citrate (pro-S)-Lyase; beta-Galactosidase; Central Nervous System Diseases; Cytoskeleton; Fumarate Hydratase; Hexosaminidases; Male; Rabbits; Sodium-Potassium-Exchanging ATPase; Spinal Cord

Lysosomal enzyme levels and nitroblue tetrazolium (NBT) reduction of phagocytes in the cerebrospinal fluid (CSF) of patients with infectious diseases of the central nervous system (CNS) were studied histochemically to evaluate the function of phagocytes. Lysosomal enzymes of acid phosphatase and beta-galactosidase were demonstrated in mononuclear phagocytes and leptomeningeal cells, but not in polymorphonuclear leukocytes (PMN) and lymphocytes. In tuberculous meningitis, more than 60% of the cells were positive for the enzymes as compared with less than 30% for other diseases. Besides, the cells which stained highly for the enzymes were often found in the CSF of tuberculous meningitis. The lysosomal enzyme levels in the cells were dependent on the nature of the infection rather than on the intensity of inflammation when judged by total cell count in the CSF. On the other hand, reduced NBT formazan was found in PMN, mononuclear phagocytes, and leptomeningeal cells. The intensity of NBT reduction by these cells correlated well with the total cell count in CSF; i.e., enhanced NBT reduction by phagocytes reflected the intensity of the inflammation in the subarachnoid space. Thus, histochemical study of phagocytes in CSF can provide useful additional aids to the diagnosis of the nature and stage of CNS infection.

Topics: Acid Phosphatase; beta-Galactosidase; Central Nervous System Diseases; Cerebrospinal Fluid; Histocytochemistry; Humans; Infections; Lysosomes; Nitroblue Tetrazolium; Oxidation-Reduction; Phagocytes

Topics: Acid Phosphatase; Aged; Basement Membrane; Central Nervous System Diseases; Desmosomes; Esterases; Female; Fibrosarcoma; Humans; Male; Meningeal Neoplasms; Microscopy, Electron; Middle Aged; Neoplasms, Radiation-Induced

A method of CSF cell culturing, based on observations of cultured cells isolated from 700 CSF specimens obtained for routine diagnostic procedures by lumbar puncture from patients who had no proven or suspected neoplastic disease, is described which enables the demonstration of proliferating mononuclear elements even when they are present in specimens with low cell count. Spread on surfaces of plastic and glass material, monocytes and histiocytes in CSF cell cultures can appear as polygonal or crescent shaped epitheloid cells, may assume spindle shapes, or transform into multinucleated giant cells. Some cells given rise to clones with different rates of proliferation, up to the formation of a monolayer. After short term culturing the cytochemical characteristics of the cells are comparable to those of the native cells. Phagocytosis in culture is possible. Cells with a high rate of proliferation can be isolated from CSF specimens in subacute non-bacterial inflammatory processes, in chronic meningitis, in the state of repair of bacterial meningitis and subarachnoid hemorrhage, after repeated lumbar punctures and other unspecific irritations such as myelography and pneumencephalography, and in the course of intrathecal cytostatic therapy.

Topics: Acid Phosphatase; Cell Count; Cell Division; Cell Survival; Cells, Cultured; Central Nervous System Diseases; Cerebrospinal Fluid; Encephalitis; Esterases; Histiocytes; Humans; Meningitis; Monocytes; Phagocytosis

Topics: Acetylcholinesterase; Acid Phosphatase; Adenosine Triphosphatases; Alkaline Phosphatase; Animals; Brain; Central Nervous System Diseases; Cholinesterases; Cresols; Esterases; Female; Histocytochemistry; Male; Mice; Organophosphate Poisoning; Phosphoric Monoester Hydrolases; Thiamine Pyrophosphate

Topics: Acid Phosphatase; Adult; Brain Neoplasms; Central Nervous System Diseases; Cerebrospinal Fluid; Child, Preschool; Epilepsy; Female; Glioblastoma; Humans; Hypersensitivity; Inflammation; Lymphoma, Large B-Cell, Diffuse; Lysosomes; Male; Mental Disorders; Neurotic Disorders; Peptide Hydrolases; Psychotic Disorders; Vascular Diseases

Topics: Acid Phosphatase; Aging; Alkaline Phosphatase; Animals; Central Nervous System; Central Nervous System Diseases; Cerebrosides; Cholesterol; Demyelinating Diseases; Disease Models, Animal; Female; Genes, Recessive; Genotype; Lipids; Male; Metabolism, Inborn Errors; Mice; Mutation; Phosphoric Monoester Hydrolases; Thyroid Function Tests; Time Factors

Topics: Acetylcholinesterase; Acid Phosphatase; Adenosine Triphosphate; Alkaline Phosphatase; Basal Ganglia; Central Nervous System Diseases; Enzymes; Esterases; Frontal Lobe; Histocytochemistry; Humans; L-Lactate Dehydrogenase; Male; Mesencephalon; Middle Aged; Monoamine Oxidase; NAD; Neuroglia; Neurons; Nucleotidases; Oxidation-Reduction; Phosphoric Monoester Hydrolases; Spinal Cord; Thiamine Pyrophosphate

Topics: Acid Phosphatase; Aphasia; Brain; Central Nervous System Diseases; Cerebral Cortex; Dementia; Deoxyribonucleases; Dihydrolipoamide Dehydrogenase; Frontal Lobe; Histocytochemistry; Humans; Lysosomes; Male; Microscopy, Electron; Middle Aged; Motor Neurons; NAD; Nerve Degeneration; Neuroglia; Oxidoreductases; Pigments, Biological; RNA; Spinal Cord

Topics: Acid Phosphatase; Alkaline Phosphatase; Animals; Central Nervous System Diseases; Haplorhini; Monkey Diseases; Neuroglia; Neurons; Poliomyelitis; Research; Spinal Cord