acid-phosphatase and Lipidoses

Topics: Acid Phosphatase; Blood Platelet Disorders; Bone and Bones; Bone Diseases; Breast Neoplasms; Electrophoresis, Polyacrylamide Gel; Erythrocytes; Female; Hematologic Diseases; Humans; Isoenzymes; Leukemia; Leukocytes; Lipidoses; Male; Primary Myelofibrosis; Prostate; Prostatic Neoplasms; Spleen; Thromboembolism

Topics: Acid Phosphatase; Amino Acid Metabolism, Inborn Errors; Amniotic Fluid; Anemia, Hemolytic, Congenital Nonspherocytic; Anemia, Sickle Cell; Carbohydrate Metabolism, Inborn Errors; Cells, Cultured; Female; Glycolipids; Glycosaminoglycans; Heterozygote; Humans; Lipid Metabolism, Inborn Errors; Lipidoses; Metabolism, Inborn Errors; Muscular Atrophy; Pregnancy; Prenatal Diagnosis; Purine-Pyrimidine Metabolism, Inborn Errors; Transferases; Xanthomatosis

Topics: Acid Phosphatase; Acid-Base Equilibrium; Chediak-Higashi Syndrome; Cytoplasm; Diffuse Cerebral Sclerosis of Schilder; Gaucher Disease; Genes, Recessive; Glycogen Storage Disease; Humans; Hydrolases; Lipidoses; Lysosomes; Metabolism, Inborn Errors; Niemann-Pick Diseases

Numerous amphiphilic cationic drugs cause generalized phospholipidosis in animals; one of these drugs is the Sandoz compound 200-125, a psychotropic agent. During a 6-month toxicity study in Charles River CD rats, a dramatic increase in foamy macrophages was seen in the lungs. A follow-up experiment was done to study the pathologic basis of these changes including a reversibility phase. Generalized phospholipidosis was induced after 4 weeks of 500 mg/kg/day of 200-125 by gavage. Characteristic pulmonary lesions consisted of extensive accumulations of large pale foamy macrophages as well as granular eosinophilic extracellular material. Lipid analyses of lungs showed marked increases in phospholipids (144%) and cholesterol esters (110%) in rats treated with 200-125. Drug metabolism studies employing 14C-labeled 200-125 showed an affinity for the drug to concentrate in the lungs and lymphoreticular system (spleen, lymph nodes) as well as in the adrenals, liver, and kidney. Reversibility of the phospholipidosis was nearly complete 4 weeks after drug withdrawal. The tissue changes were characterized by transmission and scanning electron microscopy. The potential pulmonary toxicity in humans with the amphiphiles is discussed.

Topics: Acid Phosphatase; Animals; Body Weight; Cholesterol; Female; Leukocytes; Lipidoses; Lung; Male; Neutrophils; Organ Size; Phospholipids; Psychotropic Drugs; Rats; Rats, Inbred Strains; Triglycerides

The activity of acid hydrolases was studied in serum from patients with mucolipidosis (II and III) and other lysosomal disorders. In mucolipidosis II and III all hydrolases examined except alpha-glucosidase, beta-glucosidase and acid phosphatase were greatly increased. High values for beta-galactosidase were seen in mucopolysaccharidosis types I and II, Gaucher's disease, juvenile amaurotic idiocy and metachromatic leucodystrophy. N-Acetyl-beta-glucosaminidase activity was high in mucopolysaccharidosis types I, II, III and Gaucher's disease. The activity of beta-glucuronidase was increased in mucopolysaccharidosis types I, II and III, Gaucher's disease, juvenile amaurotic idiocy and metachromatic leucodystrophy. Acid phosphatase had increased activity only in Gaucher's disease. In several lysosomal storage disorders no increased values could be found. It is suggested that high values in serum from patients with lysosomal storage disorders (not including mucolipidosis II and III) may depend upon liver cell damage, which disturbs the clearing of acid hydrolases from serum.

Topics: Acid Phosphatase; Gaucher Disease; Glycoside Hydrolases; Humans; Leukodystrophy, Metachromatic; Lipidoses; Lysosomes; Metabolism, Inborn Errors; Mucopolysaccharidoses

Topics: Acid Phosphatase; Adrenal Glands; Animals; beta-Galactosidase; Chlorphentermine; Glucuronidase; Kidney Cortex; Lipidoses; Liver; Lysosomes; Male; Mannosidases; Phentermine; Rats

Chronic treatment of rats with the amphipilic drugs chloroquine and chlorphentermine caused prominent anterior polar cataracts in virtually all rats. The basic pathologic changes underlying these cataracts were: (a) degeneration of anterior polar and sutural endings of cortical lens cells and (b) multilayered proliferation and invasion of epithelial cells into the anterior polar cortex. Ultrastructurally cortical lens cells displayed various patterns of degeneration, finally undergoing complete liquification. Liquified lens substance was phagocytosed by invading epithelial cells. Cortical lens cells and epithelial cells contained numerous lipidosis-like (lamellated) inclusions, which possessed cytochemical acid phosphatase activity. The present drug-induced lenticular alterations are interpreted as the direct or indirect consequences of a drug-induced disturbance of polar lipid metabolism in the lens.

Topics: Acid Phosphatase; Animals; Cataract; Chloroquine; Chlorphentermine; Epithelium; Female; Inclusion Bodies; Lens, Crystalline; Lipidoses; Lysosomes; Male; Phagocytosis; Phentermine; Rats

Neuronal ceroid-lipofuscinosis is characterized by pigmentary degeneration of the retina, psychomotor degeneration, epilepsy and intracellular deposition of ceroidlipofuscin. Recent reports have suggested that deficiency of peroxidase is the basic genetic defect. However, deficiency of myeloperoxidase could be demonstrated in some but not all patients; this deficiency was noted only when p-phenylenediamine (PPD) was used as hydrogen donor and could not be confirmed with guaiacol. We found that horseradish peroxidase (HR-P) oxidized PPD in the absence of added H2O2. The oxidative product of PPD showed the same absorption spectrum as the peroxidative product. The oxidation of PPD by HR-P was not inhibited by catalase or superoxide dismutase. In addition, catalase oxidized PPD in the presence of H2O2. Soluble and granular fractions obtained from human polymorphonuclear leukocytes (PMN) also oxidized PPD in the absence of H2O2. Addition of H2O2 inhibited the oxidation of PPD in some cell fractions. This inhibition could be partially eliminated by dialysis of the cell fractions. Thus, PPD is not a suitable hydrogen donor for the study of peroxidase. This may explain the variable results obtained by the previous investigators. In contrast, guaiacol did not show these undesirable characteristics. The PMN peroxidase (measured with guaiacol), catalase, beta-glucuronidase, acid and alkaline phosphatases were studied in individuals from three families with juvenile neuronal ceroid-lipofuscinosis. Family 1: an affected boy and healthy parents; all showed normal enzyme activities in both soluble and granular fractions. Family 2: two affected sisters, one healthy sib and mother, and Family 3: one affected boy; all showed reduced peroxidase activities in the granular fractions. Other enzymes were normal. The role of peroxidase deficiency in the pathogenesis of neuronal ceroid-lipofuscinosis is not clear. The basic defect of this syndrome remains uncertain.

Topics: Acid Phosphatase; Adolescent; Alkaline Phosphatase; Catalase; Cell Fractionation; Ceroid; Child; Female; Glucuronidase; Granulocytes; Humans; Leukocytes; Lipidoses; Lipofuscin; Male; Peroxidases; Syndrome

Cultured rat macrophages were used for an in vitro study of drug-induced lipidosis. Cells were exposed for 24 hours to equimolar concentrations (5 X 10(-5) and 1 X 10(-4) M) of the following amphiphilic (amphipathic) cationic drugs: chlorphentermine, amitriptyline, 1-chloro-amitriptyline, iprindole, noxiptiline, chlorpromazine. In addition the less amphiphilic drug phentermine was used. Ultrastructurally, the cytologic changes essentially consisted of formation of multilamellated cytoplasmic inclusions, which possessed acid phosphatase activity. The abnormal inclusions are interpreted to result from intralysosomal accumulation of polar lipids. Under the present in vitro conditions all drugs except phentermine, had similar potencies to induce such lysosomal alterations, quite in contrast to the great quantitative differences previously observed under in vivo conditions. The present results lend further support to a concept that regards a pronounced amphiphilic (amphipathic) character to be responsible for the lipidosis-inducing action of various cationic compounds. Cultured macrophages are suggested as a useful tool to investigate this structure-activity relationship, which under in vivo conditions may be obscured by superimposed parameters such as drug metabolism.

Topics: Acid Phosphatase; Animals; Antidepressive Agents, Tricyclic; Appetite Depressants; Chlorphentermine; Chlorpromazine; Lipid Metabolism; Lipidoses; Lysosomes; Macrophages; Microscopy, Electron; Phentermine; Rats; Structure-Activity Relationship

The single most characteristic morphological feature in I-cell disease (ICD) is the accumulation of membrane-bound vacuoles in mesenchymal cells (mainly fibroblasts). No true storage can be documented in those vacuoles. That their contents could have been dissolved during fixation or embedding remains however a possibility. Remnants consisting of a few lamellar arrays and of small amounts of fibrillo-granular material are too scarce for histochemical characterization. In hepatocytes large cells in the white pulp of the spleen and in myocardial fibers, vacuoles with fixative insoluble contents have been discovered; they are nowhere very abundant and their specificity is questionable. Because the affected fibroblastic elements represent a small fraction in any organ, most secondary biochemical abnormalities are expected to be detectable only in purely fibroblastic tissues. Our pathological study contributes to the understanding of some of the clinical features characteristic of ICD and stresses major morphological differences between ICD and the many diseases classified as mucopolysaccharidoses and mucolipidoses.

Topics: Acid Phosphatase; Alkaline Phosphatase; Brain; Cerebroside-Sulfatase; Female; Fibroblasts; Glycoside Hydrolases; Humans; Inclusion Bodies; Infant; Infant, Newborn; Kidney Glomerulus; Lipidoses; Liver; Myocardium; Neurons; Peripheral Nerves; Skin; Spleen

A pregnancy from a family in risk of I-cell disease was monitored. The fetus was diagnosed as having I-cell disease based on the findings that (1) lysosomal enzyme activities except for acid phosphatase and alpha glucosidase were clearly elevated in amniotic fluid and were reduced in cultivated amniotic fluid cells, and (2) cytoplasmic inclusions were seen in cultivated amniotic cells by phase contrast microscopy. The accuracy of prediction was confirmed by cultured skin fibroblast of the aborted fetus.

Topics: Acid Phosphatase; Adult; Amniocentesis; Amniotic Fluid; Arylsulfatases; Disaccharidases; Female; Glucuronidase; Hexosaminidases; Humans; Inclusion Bodies; Lipidoses; Lysosomes; Pregnancy

Clinical and neuropathological studies of a case of AB variant GM2-gangliosidosis have been presented. The patient was a 14 months old black female infant who had "black cherry spot" in the retinas. The total activities of beta-galactosidase and N-acetyl-beta-hexosaminidase, as well as the proportion of hexosaminidase A and B components in her serum and leukocytes were normal when the assays were carried out with artificial fluorogenic substrate. Diagnosis of GM2-gangliosidosis AB variant was established by an abnormal increase of GM2-ganglioside in the biopsied brain tissue, similar to classical Tay-Sachs disease. Her clinical manifestation appeared to be similar but somewhat milder than those of classical Tay-Sachs disease. Light microscopic features of the cerebral biopsy were also closely similar to Tay-Sachs disease and Sandhoff disease but gliosis and neuronal loss were less pronounced. Electron microscopic study revealed numerous membranous cytoplasmic bodies (MCB) and zebra bodies in neurons. In addition, varieties of large intracytoplasmic inclusions in astrocytes, a feature distinctly different from classical Tay-Sachs disease, were observed. Numerous cytoplasmic inclusions were also present in oligodendroglia, pericytes and microglial cells.

Topics: Acetylglucosaminidase; Acid Phosphatase; Astrocytes; Brain Chemistry; Capillaries; Cerebral Cortex; Female; G(M2) Ganglioside; Galactosidases; Gangliosidoses; Humans; Inclusion Bodies; Infant; Lipidoses; Neuroglia; Neurons

A 28-year-old female, who showed a floppy baby syndrome during early infancy, had a non-progressive proximal muscle weakness with easy fatiguability since childhood. Two muscle specimens biopsied at the age of 28 years revealed myriads of 1-3-mum wide abnormal spaces containing neutral fat in type I and type II fibers. Both biopsies demonstrated a type I fiber preponderance. Electron microscopy demonstrated lipid excess and normal mitochondria by simple inspection. The mitochondrial area and sarcotubular membrane profile concentration in morphometry of longitudinal sections were also normal. Cross-sections, however, revealed a slight decrease of the individual mitochondrial size and of the sarcotubular membrane profile concentration . Serum and muscle carnitine levels and the muscle carnitine palmityltransferase level were all within normal range. Besides carnitine deficiency other biochemical defects can occur in lipid storage myopathy, which represents a syndrome rather than a unique disease entity.

Topics: Acetyltransferases; Acid Phosphatase; Adenosine Triphosphatases; Adult; Carnitine; Electromyography; Female; Humans; Lipid Metabolism; Lipidoses; Male; Mitochondria, Muscle; Muscles; Muscular Diseases; Myofibrils; NADH, NADPH Oxidoreductases; Syndrome

1. In a patient with I-cell disease the activities of several acid hydrolases were elevated inplasma and reduced in cultured fibroblasts when compared with normal values. Normal activities for the enzymes were found in leucocytes. These findings agree with reports on other cases. 2. N-Acetyl-beta-D-glucosaminidase was resolved into its component forms by chromatography on microcolumns of DEAE-cellulose coupled with continuous automated assay of activity in the column effluent. Cultured skin fibroblasts from three patients showed a profound deficiency of glucosaminidase component A and a relative increase in the activity of a form eluted earlier than A. 3. In the one patient studied, the elution profile of plasma glucosaminidase was similar to that of normal plasma, but treatment with neuraminidase revealed a minor component which did not appear in control specimens. 4. Chromatographic resolution of glucosaminidase secreted by normal fibroblasts into the culture medium shoed that component A comprised two forms, a serum-type and a tissue-type, whereas only a serum-type was found in I-cell medium. 5. Different forma of alpha-L-fucosidase were shown to occur in normal plasma and fibroblasts. This is the second lysosomal hydrolase for which differences between intracellular and extracellular forms have been described and might reflect a general phenomenon. 6. The major acidic component of fucosidase from normal fibroblasts was not detected in I-cell fibroblasts. Elution profiles of fucosidase activity in normal and I-cell plasma were indistinguishable, both before and after treatment with neuraminidase. 7. On the basis of the above findings, we suggest that for several acid hydrolases there is a common biosynthetic reaction, which produces forms of these enzymes destined for incorporation into primary lysosomes rather than secretion by the cell. In cultured fibroblasts from patients with I-cell disease, the enzyme catalysing the reaction leading to the production of intracellular forms is deficient or defective, whereas the synthesis of precursor and secreted forms is unaffected.

Topics: Acetylglucosaminidase; Acid Phosphatase; alpha-L-Fucosidase; Cells, Cultured; Chromatography, DEAE-Cellulose; Disaccharidases; Glucosidases; Hexosaminidases; Humans; Leukocytes; Lipidoses; Neuraminidase

Topics: Acid Phosphatase; Cerebral Cortex; Child; Chorea; Dementia; Female; Histocytochemistry; Humans; Inclusion Bodies; Lipidoses; Male; Microscopy, Electron; Neurons; Pigments, Biological; Seizures; Speech Disorders; Syndrome

Topics: Acid Phosphatase; Biopsy; Child; Child, Preschool; Electroencephalography; Electromyography; Female; Fundus Oculi; Humans; Infant; Lipidoses; Male; Microscopy, Electron; Myotonic Dystrophy; Neurologic Examination; Ophthalmoscopy; Pedigree; Retinal Degeneration; Schwann Cells; Sural Nerve; Syndrome; Visual Acuity

Topics: Acid Phosphatase; Animals; Biopsy; Brain; Child; Child, Preschool; Consanguinity; Female; Fluorescence; Humans; Lipidoses; Lipids; Male; Microscopy, Electron; Neuroglia; Neurons; Pigments, Biological; Vacuoles

Topics: Acid Phosphatase; Animals; Centrifugation, Density Gradient; Cytoplasmic Granules; Electrophoresis, Polyacrylamide Gel; Epithelial Cells; Epithelium; Fatty Acids; Hexosaminidases; Humans; Hydrolases; Lipid Metabolism; Lipidoses; Lung; Lysosomes; Microscopy, Electron; Phosphatidylcholines; Phosphatidylethanolamines; Phospholipids; Pulmonary Surfactants; Rabbits; Sulfatases

Topics: Acid Phosphatase; Adolescent; Autopsy; Brain; Brain Chemistry; Cerebral Cortex; Cytoplasmic Granules; Electroencephalography; Female; Galactosidases; Gangliosides; Hexosaminidases; Histocytochemistry; Humans; Kidney; Lipid Metabolism; Lipidoses; Microscopy, Electron; Microscopy, Fluorescence; Muscles; Myenteric Plexus; Neurons; Osmium; Pancreas; Retina; Staining and Labeling

Topics: Acid Phosphatase; Age Factors; Apnea; Autopsy; Brain Chemistry; Diagnosis, Differential; Galactosidases; Gangliosides; Genotype; Gigantism; Hepatomegaly; Humans; Infant; Lipidoses; Male; Mucopolysaccharidosis I

Topics: Acid Phosphatase; Biopsy; Cell Nucleus; Child; Cytoplasm; Eye Movements; Female; Humans; Inclusion Bodies; Lipidoses; Lysosomes; Male; Microscopy, Electron; Pigmentation Disorders; Retinal Pigments; Schwann Cells; Spinal Nerves; Sural Nerve; Visual Acuity

Topics: Acid Phosphatase; Adolescent; Adult; Child; Child, Preschool; Galactosidases; Genotype; Heterozygote; Hexosaminidases; Humans; Infant; Isoelectric Focusing; Leukocytes; Leukodystrophy, Metachromatic; Lipidoses; Lysosomes; Mathematics; Middle Aged; Pedigree; Phenotype; Sulfatases

Topics: Acid Phosphatase; Animals; Appetite Depressants; Chlorphentermine; Inclusion Bodies; Lipidoses; Lung; Lung Diseases; Macrophages; Male; Microscopy, Electron; Phenethylamines; Phospholipids; Pulmonary Alveoli; Rats

Topics: Acid Phosphatase; Adrenal Gland Diseases; Alkaline Phosphatase; Aminoglutethimide; Animals; Atrophy; Cell Nucleus; Cytoplasm; Injections, Subcutaneous; Lipidoses; Lipids; Male; Mononuclear Phagocyte System; Rats

Ceramidase activity could not be demonstrated in the kidney and cerebellum from a deceased patient with Farber's disease, whereas the activities of six control acid hydrolase enzymes appeared normal. This enzyme defect presumably accounts for the accumulation that has been described in two patients and may represent the biochemical basis of this disorder.

Topics: Acid Phosphatase; Adult; Carbon Isotopes; Ceramides; Cerebellum; Cerebrosides; Child; Child, Preschool; Congenital Abnormalities; Female; Galactose; Galactosidases; Glucose; Glycoside Hydrolases; Heart Defects, Congenital; Hexosaminidases; Humans; Hydrolases; Infant; Infant, Newborn; Intellectual Disability; Kidney; Lipidoses; Liver Cirrhosis, Biliary; Male; Metabolism, Inborn Errors; Neuraminidase; Pigmentation Disorders; Respiratory Distress Syndrome, Newborn

Topics: Acid Phosphatase; Animals; Blood Bactericidal Activity; Bone Marrow Cells; Cattle; Cattle Diseases; Chediak-Higashi Syndrome; Cytoplasmic Granules; Humans; Kidney; Lipidoses; Lysosomes; Mice; Microscopy, Electron; Mink; Neutrophils; Peroxidases; Phagocytosis; Rodent Diseases; Staphylococcus

Topics: Acid Phosphatase; Adult; Biopsy; Bone Marrow; Brain; Child, Preschool; Female; Histocytochemistry; Humans; Infant; Lipidoses; Liver; Male; Microscopy, Electron

Topics: Acid Phosphatase; Brain; Brain Chemistry; Chromatography, Thin Layer; Female; Fetal Death; Fetal Diseases; Gangliosides; Gestational Age; Histocytochemistry; Histological Techniques; Humans; Lipid Metabolism; Lipidoses; Liver; Microscopy, Electron; Pregnancy; Spinal Cord

Topics: Acid Phosphatase; Biopsy; Brain; Child, Preschool; Cytosol; Female; Humans; Lipidoses; Lysosomes; Microscopy, Electron; Mitochondria

Topics: Acid Phosphatase; Adolescent; Child; Child, Preschool; Glycosaminoglycans; Humans; Lipidoses; Liver; Liver Diseases; Lysosomes; Male; Microscopy, Electron

Topics: Acid Phosphatase; Adult; Alanine Transaminase; Alkaline Phosphatase; Aspartate Aminotransferases; Bilirubin; Blood Protein Electrophoresis; Creatinine; Dementia; Folic Acid; Fructose-Bisphosphate Aldolase; Gait; Humans; L-Lactate Dehydrogenase; Lipidoses; Male; Movement Disorders; Pedigree; Porphyrins; Thymol

A profound deficiency (10- to 30-fold) of beta-galactosidase activity was found in tissues (liver, spleen, kidney, and brain) from two patients with generalized gangliosidosis; this deficiency is demonstrated as a failure to cleave both p-nitrophenyl-beta-D-galactopyranoside and ganglioside GM(1) labeled with C(14) in the terminal galactose. We believe that this enzymic defect is responsible for the accumulation of ganglioside GM(1) and is the fundamental enzyme defect in generalized gangliosidosis.

Topics: Acid Phosphatase; Aged; Brain; Carbon Isotopes; Child; Child, Preschool; Chromatography, Paper; Galactose; Galactosidases; Gangliosides; Glucosidases; Glycosides; Humans; Infant; Kidney; Lipid Metabolism, Inborn Errors; Lipidoses; Liver; Male; Middle Aged; Molecular Biology; Niemann-Pick Diseases; Nitrophenols; Spleen; Uracil Nucleotides

Topics: Acid Phosphatase; Adult; Brain; Brain Chemistry; Chromatography, Thin Layer; Cytoplasmic Granules; Female; Gangliosides; Histocytochemistry; Humans; Infant; Lipidoses; Liver; Male; Microscopy, Electron; Neuroglia; Neurons; Spleen

Arylsulfatase A and B have been demonstrated in preparations of human leukocytes. The level of activity of arylsulfatase A is markedly decreased in the preparations from patients with metachromatic leukodystrophy. Acid phosphatase and arylsulfatase B activities were normal. The assay of arylsulfatase A in leukocyte preparations can be useful in the diagnosis of metachromatic leukodystrophy while obviating the difficulties of current methods.

Topics: Acid Phosphatase; Adolescent; Adult; Blindness; Catechols; Child; Child, Preschool; Clinical Enzyme Tests; Diffuse Cerebral Sclerosis of Schilder; Female; Humans; Infant; Leukocytes; Lipid Metabolism, Inborn Errors; Lipidoses; Male; Muscular Dystrophies; Sulfatases; Veins

Topics: Acid Phosphatase; Adolescent; Basement Membrane; Biopsy; Cytoplasmic Granules; Female; Frontal Lobe; Gyrus Cinguli; Histocytochemistry; Humans; Lipidoses; Lipids; Microscopy, Electron; Mitochondria; Neuroglia; Neurons; Oxygen Consumption

Topics: Acid Phosphatase; Adolescent; Adult; Cerebral Cortex; Female; Galactosidases; Glucuronidase; Glycoside Hydrolases; Humans; Lipidoses; Liver; Lysosomes; Male; Spleen

Topics: Acid Phosphatase; Blood Cells; Child; Child, Preschool; Eosinophils; Female; Golgi Apparatus; Histocytochemistry; Humans; Infant; Leukocytes; Lipid Metabolism; Lipidoses; Lymphocytes; Lysosomes; Male; Microscopy, Electron; Monocytes; Niemann-Pick Diseases; Plasma Cells

Topics: Acetylcholinesterase; Acid Phosphatase; Animals; Cats; Dementia; Dihydrolipoamide Dehydrogenase; Haplorhini; Hippocampus; Histocytochemistry; Humans; L-Lactate Dehydrogenase; Lipidoses; Rabbits; Rats; Zinc

Topics: Acid Phosphatase; Brain; Cerebrosides; Chemistry Techniques, Analytical; Demyelinating Diseases; Diffuse Cerebral Sclerosis of Schilder; Diseases in Twins; Galactosidases; Histocytochemistry; Humans; Hydro-Lyases; In Vitro Techniques; Kidney; Lipid Metabolism, Inborn Errors; Lipidoses; Liver; Microscopy, Electron; Mucopolysaccharidoses; Sulfatases

Topics: Acid Phosphatase; Animals; Cholesterol; Dietary Fats; Histocytochemistry; Lipidoses; Lung Diseases; Microscopy, Polarization; Rats

Topics: Acid Phosphatase; Biopsy; Cerebral Cortex; Chromatography; Diagnosis, Differential; Electrons; Histocytochemistry; Humans; Infant; Intellectual Disability; L-Lactate Dehydrogenase; Lipidoses; Lipids; Malate Dehydrogenase; Microscopy; Microscopy, Electron; NAD; NADP; Neuroglia; Pathology; Tay-Sachs Disease

Topics: Acid Phosphatase; Brain; Child; Histocytochemistry; Humans; Infant; Lipidoses; Neurons; Tay-Sachs Disease

Topics: Acid Phosphatase; Alkaline Phosphatase; Brain; Cerebellar Diseases; Cholinesterases; Dihydrolipoamide Dehydrogenase; Electron Transport Complex IV; Glucosephosphate Dehydrogenase; Glutamate Dehydrogenase; Histocytochemistry; Histological Techniques; Humans; Hydrocephalus; Infant; L-Lactate Dehydrogenase; Lipidoses; Meningoencephalitis; Pathology; Succinate Dehydrogenase; Tay-Sachs Disease

Topics: Acid Phosphatase; Adenosine Triphosphatases; Humans; Intellectual Disability; Lipidoses; Neuroglia; Oxidoreductases; Tay-Sachs Disease

Topics: Acid Phosphatase; Gaucher Disease; Humans; Lipidoses; Phosphoric Monoester Hydrolases

Topics: Acid Phosphatase; Gaucher Disease; Lipidoses; Phosphoric Monoester Hydrolases

Topics: Acid Phosphatase; Disease; Gaucher Disease; Humans; Lipidoses; Male; Phosphoric Monoester Hydrolases; Prostate; Prostatic Diseases; Prostatism

Topics: Acid Phosphatase; Blood; Cortisone; Humans; Lipidoses; Niemann-Pick Diseases; Phosphoric Monoester Hydrolases; Pick Disease of the Brain