A Large Family with Type IV Radial Polydactyly

1998 ◽  
Vol 23 (4) ◽  
pp. 530-533 ◽  
Author(s):  
A. SEYHAN ◽  
N. AKARSU ◽  
F. KESKIN

This study examines one of the largest pedigrees with radial polydactyly type IV (uncomplicated polysyndactyly) comprising a total of 69 individuals, of whom 26 have been affected over six generations. Typical manifestations of the pedigree were bilateral radial and ulnar digital duplications, as well as syndactyly between the middle and ring fingers and the second and third toes. There was no craniofacial anomaly in any of the 17 cases examined physically. This observation suggests that radial polydactyly type IV and Greig craniofacial-synostosis syndrome with similar digital manifestations are clinically-distinct entities.

1992 ◽  
Vol 88 (3) ◽  
pp. 325-330 ◽  
Author(s):  
A. J. Richards ◽  
J. C. Lloyd ◽  
P. Narcisi ◽  
P. N. Ward ◽  
A. C. Nicholls ◽  
...  

Drug Research ◽  
2018 ◽  
Vol 68 (09) ◽  
pp. 529-535 ◽  
Author(s):  
Jamal Shamsara

AbstractMMP-2 belongs to a large family of proteases called matrix metalloproteinases (MMPs) that degrades type IV collagen, the main structural component of basement membranes and gelatin. The main pathologic role of MMP-2 overexpression is to contribute to the development of cancer through the progression of metastasis and angiogenesis. A structure-based virtual screening was employed to find new inhibitors with possible selectivity for MMP-2. The inhibitory activities of 3 inhibitors (one was not a suitable drug-like hit) among 19 purchased compounds were approved by enzyme inhibition assay. 5 hits were non-zinc-binding inhibitors of MMP-2. The results demonstrated that a computer-aided drug design could be successfully applied for discovering new MMP-2 inhibitors. We found inhibitors with new scaffolds for the inhibition of MMP-2 with some selectivity features that could be used for future lead optimization processes. According to the docked pose and MD simulation, compound 13 was expected to interact with the S1′ specificity loop of MMP-2 and had 2 π–π interactions and a stable hydrogen bond with the MMP-2 active site. The key feature of compound 13 could be used to guide the design of new non-zinc-binding inhibitors of MMP-2.


2001 ◽  
Vol 183 (10) ◽  
pp. 3176-3183 ◽  
Author(s):  
Michaela Bayer ◽  
Robert Iberer ◽  
Karin Bischof ◽  
Edith Rassi ◽  
Edith Stabentheiner ◽  
...  

ABSTRACT Protein P19 encoded by the conjugative resistance plasmid R1 has been identified as being one member of a large family of muramidases encoded by bacteriophages and by type III and type IV secretion systems. We carried out a mutational analysis to investigate the function of protein P19 and used in vivo complementation assays to test those of several P19 mutants. The results indicated that conserved residues present in the presumed catalytic center of P19 are absolutely essential for its function in conjugation of plasmid R1 and infection by the RNA phage R17. Overexpression of protein P19 in an early growth phase resulted in a massive lysis of Escherichia coli cells in liquid culture, as indicated by a rapid and distinct decrease in cell culture densities after induction. Change of the proposed catalytic glutamate at position 44 to glutamine completely abolished this effect. P19-induced cell lysis was directly shown by transmission and scanning electron microscopy. Typically, P19-overexpressing cells showed bulges protruding from the cell surfaces. Our interpretation is that these protrusions arose from a localized and spatially confined disruption of the bacterial cell wall. To our knowledge such an effect has not previously been documented for any member of the lytic transglycosylase family. From the data presented here, we conclude that protein P19 possesses the proposed localized peptidoglycan-hydrolyzing activity. This activity would be a prerequisite for efficient penetration of the cell envelope by the DNA translocation complex encoded by the conjugative plasmid.


1994 ◽  
Vol 144 ◽  
pp. 279-282
Author(s):  
A. Antalová

AbstractThe occurrence of LDE-type flares in the last three cycles has been investigated. The Fourier analysis spectrum was calculated for the time series of the LDE-type flare occurrence during the 20-th, the 21-st and the rising part of the 22-nd cycle. LDE-type flares (Long Duration Events in SXR) are associated with the interplanetary protons (SEP and STIP as well), energized coronal archs and radio type IV emission. Generally, in all the cycles considered, LDE-type flares mainly originated during a 6-year interval of the respective cycle (2 years before and 4 years after the sunspot cycle maximum). The following significant periodicities were found:• in the 20-th cycle: 1.4, 2.1, 2.9, 4.0, 10.7 and 54.2 of month,• in the 21-st cycle: 1.2, 1.6, 2.8, 4.9, 7.8 and 44.5 of month,• in the 22-nd cycle, till March 1992: 1.4, 1.8, 2.4, 7.2, 8.7, 11.8 and 29.1 of month,• in all interval (1969-1992):a)the longer periodicities: 232.1, 121.1 (the dominant at 10.1 of year), 80.7, 61.9 and 25.6 of month,b)the shorter periodicities: 4.7, 5.0, 6.8, 7.9, 9.1, 15.8 and 20.4 of month.Fourier analysis of the LDE-type flare index (FI) yields significant peaks at 2.3 - 2.9 months and 4.2 - 4.9 months. These short periodicities correspond remarkably in the all three last solar cycles. The larger periodicities are different in respective cycles.


Author(s):  
G. D. Gagne ◽  
M. F. Miller ◽  
D. A. Peterson

Experimental infection of chimpanzees with non-A, non-B hepatitis (NANB) or with delta agent hepatitis results in the appearance of characteristic cytoplasmic alterations in the hepatocytes. These alterations include spongelike inclusions (Type I), attached convoluted membranes (Type II), tubular structures (Type III), and microtubular aggregates (Type IV) (Fig. 1). Type I, II and III structures are, by association, believed to be derived from endoplasmic reticulum and may be morphogenetically related. Type IV structures are generally observed free in the cytoplasm but sometimes in the vicinity of type III structures. It is not known whether these structures are somehow involved in the replication and/or assembly of the putative NANB virus or whether they are simply nonspecific responses to cellular injury. When treated with uranyl acetate, type I, II and III structures stain intensely as if they might contain nucleic acids. If these structures do correspond to intermediates in the replication of a virus, one might expect them to contain DNA or RNA and the present study was undertaken to explore this possibility.


Author(s):  
H. Clarke Anderson ◽  
Priscilla R. Coulter

Epiphyseal cartilage matrix contains fibrils and particles of at least 5 different types: 1. Banded collagen fibrils, present throughout the matrix, but not seen in the lacunae. 2. Non-periodic fine fibrils <100Å in diameter (Fig. 1), which are most notable in the lacunae, and may represent immature collagen. 3. Electron dense matrix granules (Fig. 1) which are often attached to fine fibrils and collagen fibrils, and probably contain protein-polysaccharide although the possibility of a mineral content has not been excluded. 4. Matrix vesicles (Fig. 2) which show a selective distribution throughout the epiphysis, and may play a role in calcification. 5. Needle-like apatite crystals (Fig. 2).Blocks of formalin-fixed epiphysis from weanling mice were digested with the following agents in 0.1M phosphate buffer: a) 5% ethylenediaminetetraacetate (EDTA) at pH 8.3, b) 0.015% bovine testicular hyaluronidase (Sigma, type IV, 750 units/mg) at pH 5.5, and c) 0.1% collagenase (Worthington, chromatograhically pure, 200 units/mg) at pH 7.4. All digestions were carried out at 37°C overnight. Following digestion tissues were examined by light and electron microscopy to determine changes in the various fibrils and particles of the matrix.


Author(s):  
P.K. Simons

Glycogenosis is defined as any condition in which the tissue concentration of glycogen is increased. There are currently ten recognized variants of glycogenosis that are heritable inborn errors of metabolism. The specific enzymatic defect in each of the variants is known or at least suspected. In all cases, the enzymatic defect prevents the proper metabolism or formation of the glycogen molecule. The clinical and histologic differences between the types of glycogenosis is important to a proper diagnosis after the presence of such a condition is realized. This study was initiated to examine the ultrastructure of the rare Type IV Glycogenosis (Amylopectinosis) of which there is very little morphologic characterization in the literature.Liver tissue was obtained by needle biopsy from a 12-month-old Oriental female who was originally admitted to the hospital after observation of poor development, loss of appetite, and hepatomegaly. The majority of the tissue was fixed for light microscopy in neutral buffered formalin and processed using routine and special staining procedures (reticulin, trichrome, iron, copper, PAS, PAS-diastase and PAS-pectinase.


Author(s):  
Beverly L. Giammara ◽  
Jennifer S. Stevenson ◽  
Peggy E. Yates ◽  
Robert H. Gunderson ◽  
Jacob S. Hanker

An 11mm length of sciatic nerve was removed from 10 anesthetized adult rats and replaced by a biodegradable polyester Vicryl™ mesh sleeve which was then injected with the basement membrane gel, Matrigel™. It was noted that leg sensation and movement were much improved after 30 to 45 days and upon sacrifice nerve reconnection was noted in all animals. Epoxy sections of the repaired nerves were compared with those of the excised segments by the use of a variation of the PAS reaction, the PATS reaction, developed in our laboratories for light and electron microscopy. This microwave-accelerated technique employs periodic acid, thiocarbohydrazide and silver methenamine. It stains basement membrane or Type IV collagen brown and type III collagen (reticulin), axons, Schwann cells, endoneurium and perineurium black. Epoxy sections of repaired and excised nerves were also compared by toluidine blue (tb) staining. Comparison of the sections of control and repaired nerves was done by computer-assisted microscopic image analysis using an Olympus CUE-2 Image Analysis System.


Author(s):  
Wen-lung Wu

The mantle of bivalves has come entirely to enclose the laterally compressed body and the mantle margin has assumed a variety of functions, one of the pricipal ones being sensory. Ciliary tufts, which are probably sensory, have been reported from the mantle and siphons of several bivalves1∽4. Certain regions of the mantle margin are likely to be more or less, sensitive to certain stimuli than others. The inhalant siphon is likely to be particularly sensitive to both chemical and mechanical stimuli, whereas the exhalant siphon will be less sensitive to both. The distribution and density of putative sensory receptors on the in-and ex-halant siphon is compared in this paper.The excised siphons were fixed in glutaraldehyde and osmium tetroxide, the whole procedure of SEM study is recorded in Wu's thesis.Type II cilia cover the tips of tentacles, 6.13um. Type IV and type V cilia are found on the surface of tentacles. Type IV cilia are occasionally present at the tips of tentacles, 8 um long. They are the commonest type on the surface of tentacles. Type VI cilia occor in the internal surface of the inhalant siphon, but are not found on the surface of tentacles, 6.7-10um long.


Sign in / Sign up

Export Citation Format

Share Document