Biomedical subjects
A Feinstein
Publications and source records attributed to A Feinstein.
Infantile acropustulosis--report of a case.
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Ichthyosis-cheek-eyebrow (ICE) syndrome: a new autosomal dominant disorder.
A family presenting with ichthyosis vulgaris, prominent full cheeks, sparse lateral eyebrows and other craniofacial and musculoskeletal defects is described in detail. This constellation of physical findings represents a new syndrome, transmitted in an autosomal dominant fashion. For reasons of simplicity it has been termed the I (ichthyosis), C (cheek), E (eyebrow) syndrome.
Ichthyosis-cheek-eyebrow syndrome (ICE(skin))
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Anti-progesterone monoclonal antibody affects early cleavage and implantation in the mouse by mechanisms that are influenced by genotype.
Pregnancy was blocked by anti-progesterone monoclonal antibody in two inbred (BALB/cJ, CBA/Ca) but to a lesser degree in an F1 hybrid (CBA/Ca male X BALB/cJ female) or an outbred (Tuck's no. 1) stock of mice when antibody was injected intraperitoneally (i.p.) at 32 h post coitum (p.c.) using a dosage of 9.5-10.9 nmol. This different antifertility effect could not be explained solely by altered tubal transport in inbred mice since the rate of transport was slightly accelerated in one stock (BALB/c) but not in another (CBA). In crossbred mice tubal transport was not significantly altered by antibody treatment. At Day 3 (54-58 h p.c.), the majority of embryos in control mice were at the 4-cell and 8-cell to morula stages in inbred and crossbred stock, respectively, but after antibody treatment they were mainly at the 4-cell stage in all 4 stocks. At Day 4 (78-82 h p.c.) the majority of embryos in control females had reached the blastocyst stage in all stocks, whereas after antibody treatment they had reached this stage in crossbred stock and relatively few had progressed so far in inbred stock. The results indicate that there are two events in early gestation which are susceptible to passive immunization with anti-progesterone monoclonal antibody. The first of these occurs during cleavage shortly after the 4-cell stage when embryo development was arrested in two inbred stocks of mice. Antibody effects on cleavage were not direct since embryos cultured in the presence of high concentrations of antibody, or antibody saturated with progesterone, continued to develop in the normal way and formed blastocysts. The second event is the onset of implantation, an effect also influenced by genotype. The decidual cell reaction induced by intraluminal oil injection was blocked by antibody injected at 8 or 32 h p.c. in BALB/c females, but only when injected at 8 h, and not at 32 h p.c., in F1 hybrid females. The results show that there is a greater resistance in two crossbred stocks compared with two inbred stocks to the effects of passive immunization against progesterone in early pregnancy.
Skin tags: a cutaneous marker for diabetes mellitus.
Two hundred and sixteen non hospitalized patients with skin tags (ST) were studied for the presence of diabetes mellitus (DM) and obesity. Overt DM was found in 57 (26.3%) patients and impaired glucose tolerance test was found in 17 (7.9%) patients. Sixteen new cases of DM were found among this group. All the diabetic patients in the study population had non-insulin dependent DM. Sixty-two (28.7%) of the patients were obese. No correlation was found between the localization, size, colour and number of the ST and the presence of DM. Our study indicates that ST are not associated with increased incidence of obesity compared to the general population. On the other hand, ST are associated with impaired carbohydrate metabolism, and may serve as means for identifying patients at increasing risk of having DM.
Analysis of an anti-progesterone antibody: variable crystal morphology of the Fab' and steroid-Fab' complexes.
Anti-progesterone monoclonal antibodies are being used for structural studies of antibody-antigen interaction, for their ability to block pregnancy shortly after fertilization, and for hormone immunoassay. A mouse anti-progesterone monoclonal Fab' fragment has been crystallized in its native form and co-crystallized with seven different, but structurally related, steroids. The crystals show interesting preferences in their crystal morphology, depending on the bound steroid ligand. The X-ray crystallographic analysis of this Fab', complexed with a series of related steroid ligands, should reveal details of the chemistry of antibody-antigen union and provide insights into how steroids interact with proteins.
Genetic disorders associated with severe alopecia in children: a report of two unusual cases and a review.
Moderate to severe alopecia in children may be due to a genetic disorder. This paper reviews the heritable causes of alopecia in children and gives a detailed account of two affected unrelated children. One has alopecia universalis while the other has alopecia postulated to be due to a new disorder of genetic etiology. The article concludes that for purposes of genetic counseling and prognosis it is crucial that a correct diagnosis be made.
Anti-progesterone antibodies: specificity, structure, idiotypes and anti-idiotypic manipulation.
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Identification and isolation of two pentraxins from bovine serum.
Two distinct pentraxin proteins were isolated from bovine serum by calcium-dependent affinity chromatography on high pyruvate agarose gel. One of these proteins cross-reacted specifically with certain rabbit anti-human CRP antisera and was therefore designated as bovine CRP. The other cross-reacted specifically with a sheep anti-human SAP antiserum and was therefore designated as bovine SAP. Although the mixture of these two pentraxins was not resolved by gel filtration chromatography, they were separated by solid phase absorption of the CRP with immobilized rabbit anti-human CRP antibodies. Their identity as pentraxins was confirmed by their electron microscopic appearance. Bovine CRP was composed of a single type of non-glycosylated subunit whilst bovine SAP contained two major types of glycosylated subunits and a minor polypeptide, the glycosylation of which was not determined. Serum concentrations were in the range of 5-40 mg/l and neither protein behaved as an acute phase reactant. No bovine serum protein undergoing calcium-dependent binding to phosphoryl choline or pneumococcal C-polysaccharide was obtained.
Pemphigus foliaceus coexisting with toxic, multinodular goiter.
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Treacher-Collins syndrome and neurofibromatosis.
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Anti-fertility effect of passive immunization against progesterone is influenced by genotype.
The anti-fertility effect of a monoclonal progesterone antibody injected i.p. 32 h after mating is influenced by genotype since a single dose of 5.7 nmol immunoglobulin G successfully blocked the establishment of pregnancy in BALB/c but not in F1 hybrid mice (CBA male X BALB/c female). Progesterone concentrations in circulation were significantly higher at days 3 and 4 after mating in F1 females compared with those of the BALB/c stock. Moreover, the pattern of mitotic activity in the endometrium after passive immunization differed between the two genotypes. In treated BALB/c mice there was no increase in mitotic activity in stromal cells at days 3, 4 and 5 after mating (in contrast to BALB/c control females in which the number of stromal mitoses increased sharply). In F1 females there was a transient effect of antibody at day 3 (no increase in stromal mitoses but enhanced mitotic activity in the glandular epithelium compared with F1 control females), and subsequently a normal increase in mitotic divisions in stromal cells. The hypothesis is proposed that passive immunization against progesterone at 32 h after mating will only block the establishment of pregnancy in genotypes in which there is a gradual, rather than a steep rise in circulating progesterone concentrations during the preimplantation period. In F1 mice, high concentrations of circulating progesterone at days 3 and 4 of pregnancy apparently over-ride the effect of antibody and facilitate the normal development of stromal mitotic activity associated with the onset of implantation.
Anti-idiotypic sera against monoclonal anti-progesterone antibodies: production in rabbits and rats and characterization of specificity.
Antisera were raised in rabbits and rats against three mouse monoclonal anti-progesterone IgG1 antibodies. Anti-idiotypic antibodies were isolated from rabbit sera by successive passage over immunoadsorbent columns of normal mouse Ig and the specific immunizing monoclonal, with elution from the latter. Radioimmunoassays for anti-idiotype and free idiotype were established, enabling detection of idiotype in sera of mice immunized with progesterone-BSA conjugate. Binding of rabbit anti-idiotype to anti-progesterone monoclonals was partially inhibitable by free progesterone-hemisuccinate or progesterone-ovalbumin conjugate. While showing considerable specificity for their respective inducing monoclonals, the anti-idiotypes also cross-reacted in varying degrees with the other anti-progesterone monoclonals, demonstrating the presence of IdI and IdX determinants. The patterns of cross-reactivity showed some correlation with the relative isoelectric points and combining-site specificities of the anti-progesterones. The specificities of rat and rabbit anti-idiotypes were similar, but not identical.
Antibody density on rat red cells determines the rate of activation of the complement component C1.
It is a common observation that there is variability in the rate of activation of C1, the first component of complement, when bound to immune complexes. The cause of this variation has been investigated with experiments designed to assess separately the effect of antibody, antigen and C1 density. Using 125I-labeled C1 and a rat monoclonal antibody specific for the class I antigen, it has been found that the rate of activation is primarily dependent on antibody density on the cell surface and not on antigen or C1 density. This finding supports the suggestion that direct contact between the C1r2C1s2 subcomponent of C1 and antibody may be required for potentiation of C1 activation.
Effector functions of a monoclonal aglycosylated mouse IgG2a: binding and activation of complement component C1 and interaction with human monocyte Fc receptor.
Aglycosylated monoclonal anti-DNP mouse IgG2a produced in the presence of tunicamycin was compared with the native monoclonal IgG2a with respect to its ability to interact with the first component of complement, C1, and to compete with human IgG for binding to human monocyte Fc receptors. The aglycosylated IgG2a was found to bind subcomponent C1q with an equivalent capacity to the native IgG2a, but the dissociation constant was found to be increased three-fold. When activation of C1 by the glycosylated and aglycosylated IgG2a was compared, the rate of C1 activation by the aglycosylated IgG2a was reduced approximately three-fold. In contrast aglycosylation was accompanied by a large decrease (greater than or equal to 50-fold) in the apparent binding constant of monomeric IgG2a to human monocytes. The data suggest that the aglycosylated IgG2a has a structure which differs in the CH2 domain from the native IgG2a, and that the heterogeneous N-linked oligosaccharides of this monoclonal IgG2a which occur at a conserved position in the CH2 domain play a role in maintaining the integrity of its monocyte-binding site. This lack of monocyte binding may result either from a localized conformational change occurring in a single CH2 domain or from an alteration in the CH2-CH2 cross-domain architecture which is normally structured by a pair of opposing and interacting oligosaccharides. The minimal changes in C1q binding and C1 activation suggest that the oligosaccharides are, at most, indirectly involved in these events.
Lichen aureus occurring in childhood.
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The effects of binding mouse IgA to dinitrophenylated Salmonella typhimurium on physicochemical properties and interaction with phagocytic cells.
The binding of mouse IgA antibody to DNP-conjugated bacteria enhanced the hydrophobic interaction when measured in dextran-poly(ethyleneglycol) two-phase systems and hydrophobic interaction chromatography. Similarly the interaction with polymorphonuclear leukocytes in vitro increased. In contrast, the rate of clearance of IgA-reacted bacteria from the blood stream of mice, after intravenous injection, was reduced under the conditions tested. Our data showed, with respect to increased hydrophobic interaction and opsonization, that binding of IgA antibody to bacteria produced less pronounced effects than those of IgG antibody binding. In contrast, secretory IgA antibody binding has been shown to reduce hydrophobic interaction and phagocytosis. Thus, serum IgA antibody has properties intermediate to IgG and SIgA with respect to hydrophobicity and opsonization.