Lysosomal sialidase deficiency in sialidosis with partial β-galactosidase deficiency

We analyzed the subcellular localization of sialidases in human lymphocytes from a patient with adult type sialidosis with partial β-galactosidase deficiency and normal controls. Sialidase activities were measured with α,2 → 3 NeuAc-lactitol, 4-methylumbelliferyl-NeuAc and G_M3 ganglioside as substrates. Sialidases in the lysosomes were sonication-labile and hydrolyzed mainly hydrophilic substrates such as NeuAc-lactitol and 4-methylumbelliferyl-NeuAc, but hydrolyzed subsidiarily G_M3 ganglioside. On the other hand, sialidases in the plasma membrane were sonication-stable and hydrolyzed both hydrophilic substrates and G_M3 ganglioside. In sialidosis with partial β-galactosidase deficiency, the sialidases of the lysosomes showed 3–5% activity toward hydrophilic substrates and 25% activity toward G_M3 ganglioside as compared with sialidase activities of the controls. However, there are no differences in the activities of the sialidases in the plasma membrane. These results demonstrate that the essential defect in this disease is the deficiency of a lysosomal sialidase.     

Boron deficiency in maize

Boron (B) deficiency depresses wheat, barley and triticale yield through male sterility. On the basis of field responses to B fertilization, maize (Zea mays L.) is affected by B deficiency in five continents. In a series of sand culture trials with maize subject to B0 (nil added B) and B20 (20???M added B) treatments, we described how B deficiency depressed maize grain yield while showing an imperceptible effect on vegetative dry weight. With manual application of pollen to the silk of each plant, B0 plants produced 0.4 grain ear^?1 compared with 410 grains ear^?1 in B20 plants. Symptoms of B deficiency was observed only in B0 plants, which exhibited symptoms of narrow white to transparent lengthwise streaks on leaves, multiple but small and abnormal ears with very short silk, small tassels with some branches emerging dead, and small, shrivelled anthers devoid of pollen. Tassels, silk and pollen of B0 plants contained only 3?C4?mg B kg^?1 DW compared with twice or more B in these reproductive tissues in B20 plants. A cross-fertilization experiment showed that, although the tassels and pollen were more affected, the silk was more sensitive to B deficiency. Pollen from B20 plants applied to B0 silk produced almost no grains, while pollen from B0 on B20 silk increased the number of grains to 37% of the 452 grains plant^?1 produced from B20 pollen on B20 silk. Therefore, the silk of the first ear may be targeted for precise diagnosis of B status at maize reproduction, for timely correction by foliar B application, and even for B-efficient genotype selection.     

Androgen deficiency in women

Androgens are defined as the steroids having a binding affinity of the androgen receptor. In the reproduction age a daily production of testosterone is equally divided between the ovaries and adrenal and local tissue conversion of androstenedione and DHEA. After menopause the 80% of testosterone is produced in ovaries, but majority of precursors for peripheral conversion is adrenal origin. Androgen receptors are present throughout in the body; over 200 cellular actions of androgens have been described. Androgenic action is determined by quantitative level of the androgen present in the circulation, its degree of binding to proteins, the degree of interconversion to other androgens and estrogens, and the biological potency and androgen receptor binding affinity of the androgen. The most common clinical symptoms of androgen deficiency are the reduction of sex motivation, sex fantasy, sex enjoyment, sex arousal, vaginal vasocongestion, but also reduction of pubic hair, bone mass, muscle mass, worsening of quality of life (mood, affect, energy), more frequent vasomotors symptoms, insomnia, depression, headache. All these signs and symptoms can be multifactorial. Most common conditions associated with hypoandrogenism in women are hypothalamic-pituitary abnormalities, lack or insufficiency of ovaries, adrenal insufficiency, glucocorticoid therapy, exogenous estrogen administration. Besides the clinical picture the free testosterone measuring is important for diagnosis. The method of choice of this measure is equilibrium dialysis assay. Despite of clinical importance of androgen insufficiency in women, none of methods of androgen substitution is approved by FDA.     

Studies in the diagnosis of mineral deficiency; manganese deficiency in wheat

At fourteen sites where winter wheat was growing commercially, twelve of which were known or suspected to be deficient in manganese, a spray of manganese sulphate was applied late in the shooting stage of development, and the effect on yield of grain estimated. Samples of certain organs of unsprayed wheat plants were gathered on three occasions between tillering and ear emergence, and samples of weed leaves on the same occasions and after harvest; these samples were analysed spectrographically for manganese.
The results indicate that analysis of the wheat plant can be used to forecast its response to a manganese sulphate spray, and the following tentative limiting values for manganese content are put forward, above which no increase in grain yield as a result of treatment may be expected: (a) during tillering, 34 p.p.m. manganese in dry matter of lower leaf blades; (b) just prior to ear emergence, 36 p.p.m. manganese in dry matter of stems. The highest correlation obtained between response and manganese content was that with the values for stem samples gathered just prior to ear emergence.
Analysis of weed samples can only be expected to indicate gross differences in the manganese status of different sites.     

Selenium deficiency in Crustacea

Summary The effect of selenium deprivation onDaphnia magna was examined under controlled rearing conditions in a synthetic culture medium. After three generations, fertility was significantly reduced in deprived (Se^–) animals. Growth and mortality of parent daphnids and development of parthenogenetic eggs were not affected during this period. In the fourth generation Se^– daphnids rejected parts of their second antennae. At the ultrastructural level antennal muscle tissue was severely affected. Animals deprived of selenium had mitochondria and sarcoplasmic reticulum with myelin-like alterations. Giant lysosomes were present and complete lysis of muscle fibrils was observed in antennal muscle cells. These alterations are characteristic features of peroxidic damage in tissues. This interpretation is consistent with the function of selenium as a constituent of the enzyme glutathione peroxidase which protects cells from peroxidation. Selenium should be included in synthetic culture media for daphnids.Abbreviations GSH-Px selenium dependent glutathione peroxidase - Se⁺/Se^– selenium supplemented (control)/selenium deprived animals - SOD Superoxide dismutase - SR sarcoplasmic reticulum     

Selenium deficiency in yugoslavia

Data on selenium (Se) deficiency in Yugoslavia are presented. The results include Se content of soil, cereal crops, and garlic grown in these soils, and human serum and scalp hair from several towns and regions. All data indicate a serious Se deficiency: soil (n=140), the mean value of 200±69.6 μg/kg Se; wheat, (58) = 20.5 ± 12.4 μg/kg; corn, (79) = 13.7 ± 13.6 μg/kg; and garlic, (66) = 13.7 ± 17.1 μg/kg Se. Analyses of human tissue show a very low Se status of the Yugoslav population: serum, (n=875) = 50.0 ± 18.0 μg/L and scalp hair, (388) = 94 ± 16 μg/kg Se. In some regions, Se contents of grain, garlic, and human serum and hair are approaching those in the low-Se belt in China. It is assumed that very low Se status of a human population could be a risk factor in the development of Balkan Endemic Nephropathy (BEN) and in a high incidence of urinary tract tumors (UTT) in endemic areas. A portion of these results were presented at the International Symposium on Selenium, Belgrade, May 12–15, 1991, Abstracts p.1, Serbian Academy of Sciences and Arts.     

Radial ray deficiency and ulnar ray deficiency in two sibs.

A kindred with a diabetic mother is described with one sibling with a radial deficiency (type 1 radial dysplasia (Bayne) with a hypoplastic thumb type 3 of Blauth) and one with an ulnar ray deficiency (type 2 of Ogden or type 1 of Swanson). A metabolic cause is probable, although the diabetes was well controlled during pregnancy.     

Pathological consequences of copper deficiency and cobalt deficiency

Aspects of the pathology of copper deficiency in several species, and cobalt deficiency in sheep, are summarized. An attempt is made to interpret morphological changes in copper-deficient animals in terms of biochemical defects. The common denominator may be mitochondrial lesions, with a generalized effect on energy-dependent synthetic functions of the cell. In copper deficiency, such defects can be attributed to depletion of copper-dependent enzymes, while deficiency of cobalt in ruminants is, in effect, deficiency of vitamin B12. The pathological consequences of vitamin B12 deficiency form a syndrome, notable features of which are neurological and muscular lesions, in which the metabolic consequences of hepatic damage may play a significant role.     

Nitrogen-induced boron deficiency in lucerne

Summary In a glasshouse experiment with a boron deficient soil the application of nitrogen was found to decrease the boron concentration and boron uptake by lucerne (Medicago sativa). Without added boron, nitrogen applications killed the lucerne, probably by inducing severe boron deficiency. With added boron, the lowest rate of nitrogen application increased lucerne yield but further additions depressed yields. The effect was due to a physiological interaction rather than an effect of the nitrogen on the availability of the boron in the soil.