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Biomedical subjects

B Glick

Publications and source records attributed to B Glick.

At least 37 records · Page 2Linked to original sources
PubMed1992

Familial hypotonia of childhood caused by isolated 3-methylcrotonyl-coenzyme A carboxylase deficiency.

Hypotonia was the initial symptom in four siblings from a nonconsanguineous Tunisian-Jewish family. Plasma carnitine was severely deficient, and urinary organic acid analysis revealed increased excretion of 3-hydroxyisovaleric acid and 3-methylcrotonylglycine. 3-Methylcrotonyl-coenzyme A carboxylase activity was reduced in skin fibroblasts; pyruvate carboxylase and serum biotinidase activities were normal. We conclude that 3-methylcrotonyl-coenzyme A carboxylase deficiency should be added to the list of metabolic causes of familial hypotonia of childhood.

Carbon-Carbon Ligases↗
PubMed1992

The effect of cyclophosphamide on bursal and splenic dendritic cells.

The effect of in ovo and posthatch administration of cyclophosphamide, an immunosuppressive drug, on avian dendritic cells was examined in New Hampshire chickens. The Small Bursa line of New Hampshire chickens was more sensitive to the toxic effects of this drug than the line from which it was selected, the Lester J. Dreesen line. The bursal secretory dendritic cells, which may effect B cell differentiations, were left intact and appeared more prominent after treatment. The ellipsoid-associated cells, the dendritic cells of the spleen that bind and carry antigen, became vacuolated after treatment, but normal ellipsoid-associated cells were found within 1 wk. Ellipsoid-associated cells were able to bind and move carbon away from the ellipsoid areas of the spleen 5 wk after in ovo treatment with cyclophosphamide. However, the ability of treated birds to mount an antibody response to SRBC was compromised. The present study revealed that bursal dendritic cells were resistant to cyclophosphamide treatment during embryogenesis and the same embryonic treatment did not influence the migrating capability of ellipsoid-associated cells.

Animals↗
PubMed1992

Plasma cells expressing immunoglobulins M and A but not immunoglobulin G develop an intimate relationship with central canal epithelium in the harderian gland of the chicken.

In the Harderian gland of the chicken, the epithelial and plasma cell relationships were studied by light and electron microscopy and immunohistochemical methods. In the wall of the central canal a dark epithelial cell was identified that had long branching cell processes. An anticytokeratin monoclonal antibody demonstrated that the dark cells provided an extremely large contact area for the plasma cells. Although IgM-, IgG-, and IgA-producing plasma cells were present in the Harderian gland, only IgM- and IgA-positive cells were capable of a distinct relationship with dark epithelial cells. The surface of the primary branches contained scattered IgA deposits whereas the epithelial cells of the secondary branches possessed IgA along the lateral cell membrane but not on the surface. Anti-IgA and anti-cytokeratin antibodies produced a similar staining pattern in the acini and secondary branches. Taken together, these observations suggest that IgA secretion is a function of secondary branches and that intracellular transport is influenced by the cytoskeletal system.

Animals↗
PubMed1992

Dynamic changes in the intermediate filaments of the epithelial cells during development of the chicken's bursa of Fabricius.

The development of bursal follicles and the differentiation of the follicle-associated epithelium and interfollicular epithelium were studied immunohistochemically using monoclonal anti-vimentin and anti-cytokeratin antibodies. In 10-day-old embryos the entodermal and cloacal epithelia coexpressed vimentin- and cytokeratin-intermediate filaments. Both undifferentiated and differentiated bursal surface epithelium simultaneously expressed vimentin- and cytokeratin-intermediate filaments during the entire period of embryogenesis. Vimentin expression in reticuloepithelial cells was related to bursal cell differentiation but was not linked to immune function. Sequential loss of vimentin from interfollicular epithelium, follicle-associated epithelium, and reticuloepithelial cells may reflect sequential acquisition of maturity in these three compartments. The presence of cytokeratin-intermediate filaments suggested that follicle-associated epithelium was not of mesenchymal origin. Testosterone treatment did not influence the vimentin and cytokeratin filament expression in the epithelial cell.

Animals↗
PubMed1992

Nonketotic hyperglycinemia presenting with pin-point pupils and hyperammonemia.

We describe two siblings who presented with lethargy, decreased sucking, respiratory failure and seizures in their first days of life. Pin-point pupils were noted in both siblings. Amino acid analysis revealed elevated concentrations of glycine in plasma and cerebrospinal fluid (CSF), with abnormal CSF/plasma ratios, compatible with hyperglycinemia. Urine organic acid analysis was unremarkable. Hyperammonemia was found in both siblings, but this subsided within 24-72 h. We suggest that pin-point pupils may be an additional presenting sign of nonketotic hyperglycinemia and, if looked for early enough, hyperammonemia may be found in this disorder.

Ammonia↗
PubMed1992

Non-lymphoid cells produce IgA-like substance in the corticomedullary zone of the chicken thymus. IgA-like substance in non-lymphoid cells.

Anti-IgA monoclonal antibody identified two different types of cells at the corticomedullary border of the chicken thymus. While cell Type I might be a plasma cell, Type II was an epithelial cell with intracytoplasmic and intracystic reaction products. The alpha chain reaction product of the epithelial cell was not recognized by anti lambda light chain antibody suggesting that the product was not a complete immunoglobulin A. It is proposed that the product of some corticomedullary epithelial cells may contribute to the induction of T alpha cells.

Animals↗
PubMed1992

The ruptured abdominal aortic aneurysm in a community hospital. A 5-year study.

The authors report on a recent 5-year experience with the treatment of ruptured abdominal aortic aneurysms in a community hospital located in a frequently visited tourist center. Their mortality rate of 43 per cent compares favorably with accepted mortality rates from large teaching institutions. Several known complications of this condition occurred in the authors' series. The most ominous complications that carried the highest associated mortality rates, if the patient did not succumb in the operating room, were acute tubular necrosis (75% associated mortality) and bowel infarction (100% associated mortality). Most important, physicians must have a high index of suspicion to make the diagnosis early. As reported in other series, prolonged hypotension and blood loss has a direct correlation with high mortality.

Age Factors↗
PubMed1991

An Ig-A-like substance in the chicken's pineal.

Immunohistochemistry revealed an Ig-A-like substance on the luminal surface of the pineal follicles and in the parafollicular layer. This substance was observed around 1 week of age and disappeared by 8 weeks at the time when the transformation of the follicular pattern leads to an adult-type pineal tissue.

Animals↗
PubMed1991

Historical perspective: the bursa of Fabricius and its influence on B-cell development, past and present.

The bursa of Fabricius has a history and a future. The history included its description by Hieronymus Fabricius and the discovery in the 1950s of its pivotal role in humoral immunity. The apparent obligate role of the bursa in B-cell development was modified by research in the 1960s and 1970s which described the synthesis of immunoglobulin in bursaless birds and led to the concept of extra bursal sites. Then in the 1980s, supported by the research of the past 25 years and the new technology, the obligate role of the bursa in orchestrating the V-gene repertoire-antibody diversity was revealed. Microenvironmental studies in the 1970s and 1980s announced the importance of bursal epithelium, secretory dendritic cells, and other reticuloepithelial cells in interpreting the ontogeny of B-cell differentiation. The past history of the bursa will be remembered for its contribution to present and future research and the present and future will be promising if the experiences of the past are not forgotten.

Animals↗
PubMed1991

Protein import into mitochondria: two systems acting in tandem?

Mitochondria can import proteins from the cytoplasm at sites where the two mitochondrial membranes are closely apposed. However, each of these membranes contains a distinct protein translocation channel. Recent evidence suggests that the two types of channel are not permanently coupled, but may dissociate in a reversible manner. This reversible interaction is probably essential for intramitochondrial sorting proteins.

Journal Article↗
PubMed1991

Infantile spinal muscular atrophy (SMA) and multiple congenital bone fractures in sibs: a lethal new syndrome.

Acute infantile spinal muscular atrophy (SMA type I, Werdnig-Hoffmann disease) has generally been accepted as an autosomal recessive disorder. However, several investigators have noted a slightly increased male to female ratio. We describe here a family with two affected male sibs who had a form of acute infantile SMA with congenital bone fractures, whose parents were first cousins. Pedigree analysis strongly suggested autosomal recessive inheritance, but X linked recessive inheritance could not be ruled out. In view of the heterogeneity of the SMAs, and the distinct clinical features found in our patients, we suggest that their infantile SMA might well be a distinct entity. We suggest that SMA I with congenital contractures and bone fractures appears to be a recognisable disorder that can be distinguished from the more common classic form of SMA I.

Arthrogryposis↗
PubMed1991

Endogenous peroxidase- and vimentin-positive cells accumulate at the corticomedullary border of the chicken thymus.

The granulopoietic activity of the thymus and bursa of Fabricius was analyzed using an anti-vimentin monoclonal antibody and endogenous peroxidase reaction to detect possible hemopoietic precursors and functionally mature cells, respectively. Endogenous peroxidase-positive cells (EPC) and vimentin-positive cells (V9) were located at the corticomedullary zone of the thymus. The cells were grouped, suggesting their clonal expansion. The first EPC appeared in the thymus on Day 15 of embryonic age, 2 and 3 days later than in the bursa of Fabricius and spleen, respectively. They were intermingled with thymocytes. Although the bursal EPC disappeared by Day 7 after hatching, the number of thymic EPC continued to increase. The thymic EPC and V9 cells persisted for more than 12 wk of age. The V9 cells were present either as single cells or grouped cells in the 15-day-old embryos. The V9 cells represented a distinct cell population of the corticomedullary border that may cooperate with the EPC.

Animals↗
PubMed1991

Effects of several selected odorants on the sodium- and potassium-dependent adenosine triphosphatase activities of two different chicken olfactory tuberinals.

The NaK-adenosine triphosphatase (ATPase)-rich nerve-ending particle preparations (B fractions) of the epithelial tissue of chicken olfactory tubercle and the olfactory main concha were isolated by differential centrifugation. These tissues were exposed to the following odorants: 2-nonanone, 1-nonanol, 1-octanol, (+)2-octanol, and (-)2-octanol. There was a significant stimulation of NaK-ATPase by 2-nonanone (1 x 10(-3) M) in the B fraction of the olfactory tubercle and olfactory main concha. The NaK-ATPase increased significantly in the B fraction response of the olfactory main concha with 1 x 10(-3) M of 1-octanol but NaK-ATPase declined significantly in the presence of 1 x 10(-3) M of 1-nonanol. Both optical and structural isomers of odorants were shown to elicit different responses in NaK-ATPase activity. It is proposed that interactions between the odorant molecules and the membrane bound NaK-ATPase complex lead to recognition of odor by chickens.

1-Octanol↗
PubMed1990

Mitochondrial protein import.

Most polypeptides of mitochondria are imported from the cytosol. Precursor proteins contain targeting and sorting information, often in the form of amino-terminal presequences. Precursors first bind to receptors in the outer membrane. Two putative import receptors have been identified: a 19-kilodalton protein (MOM19) in Neurospora mitochondria, and a 70-kilodalton protein (MAS70) in yeast. Some precursors integrate directly into the outer membrane, but the majority are translocated through one or both membranes. This process requires an electrochemical potential across the inner membrane. Import appears to occur through a hydrophilic pore, although the inner and outer membranes may contain functionally separate translocation machineries. In yeast, a 42-kilodalton protein (ISP42) probably forms part of the outer membrane channel. After import, precursors interact with "chaperonin" ATPases in the matrix. Presequences then are removed by the matrix protease. Finally, some proteins are retranslocated across the inner membrane to the intermembrane space.

Animals↗
PubMed1990

The role of avian macrophages in the production of avian lymphokines.

Lymphocyte inhibitory factor (LyIF) and lymphocyte chemotactic factor (LCF) were produced by sensitized thymic (T) and bursal (B) cells and thymic (T) cells, respectively. The passage of sensitized T and B cells through a Sephadex G-10 column abrogated the ability of T cells to produce LyIF and LCF while B cells remained capable of producing LyIF. The return of macrophages to the adherent cell depleted population reconstituted the T cells' ability to produce LyIF and LCF. These data suggest a greater macrophage dependency of thymic cells than bursal cells in the production of lymphokines.

Animals↗
PubMed1990

Effect of the progestogen, allylestriol, on the bursal development of the chicken.

Fertile eggs were dipped on the fifth day of incubation into varying concentrations of allylestriol (AE), a synthetic progesterone, and testosterone propionate (TP). Concentrations of AE higher than 0.1% inhibited hatchability. The AE inhibited the differentiation of the bursal mesenchyme and follicular development and subsequently resulted in bursectomy. The AE caused bursectomy in 40-50 times lower concentrations than did TP. An unusual lymphocyte accumulation occurred around the postcapillary venules in the bursal mesenchyme of AE-treated birds. This observation suggested that inhibition of mesenchyme differentiation may lead to a modification in bursal function. The AE modification of bursal development was compared to those produced by TP. We demonstrated that AE caused immunosuppression of the B-cell system.

Animals↗