Multiple molybdoenzyme deficiencies due to an inborn error of molybdenum cofactor metabolism: Two additional cases in a new family

1983 ◽  
Vol 6 (S2) ◽  
pp. 95-96 ◽  
Author(s):  
A. Munnich ◽  
J. M. Saudubray ◽  
C. Charpentier ◽  
H. Ogier ◽  
F. X. Coudé ◽  
...  
Keyword(s):  
Inborn Error ◽  
Molybdenum Cofactor ◽  
New Family ◽  
Cofactor Metabolism

Molybdenum Cofactor Deficiency: Another Inborn Error of Metabolism With Neonatal Onset

PEDIATRICS ◽  
1988 ◽  
Vol 82 (3) ◽  
pp. 521-521
Author(s):  
BARBARA K. BURTON
Keyword(s):  
Inborn Error ◽  
Molybdenum Cofactor ◽  
Inborn Error Of Metabolism ◽  
Inborn Errors ◽  
Oxidase Deficiency ◽  
Ectopia Lentis ◽  
Molybdenum Cofactor Deficiency ◽  
Sulfite Oxidase Deficiency ◽  
Eye Examination ◽  
Cofactor Deficiency

In Reply.— Matsuo and co-workers correctly point out that molybdenum cofactor deficiency may present in the neonatal period and, therefore, add yet another disorder to the list of inborn errors of metabolism affecting the neonate. Hypouricemia may be a clue, as noted, and ectopia lentis, as seen in isolated sulfite oxidase deficiency, is an additional important finding. The importance of a careful eye examination in infants suspected of having an inborn error of metabolism is again emphasized.

scholarly journals A widespread riboswitch candidate that controls bacterial genes involved in molybdenum cofactor and tungsten cofactor metabolism

2008 ◽  
Vol 68 (4) ◽  
pp. 918-932 ◽  
Author(s):  
Elizabeth E. Regulski ◽  
Ryan H. Moy ◽  
Zasha Weinberg ◽  
Jeffrey E. Barrick ◽  
Zizhen Yao ◽  
...  
Keyword(s):  
Molybdenum Cofactor ◽  
Cofactor Metabolism ◽  
Bacterial Genes ◽  
And Tungsten

Molybdenum Cofactor Deficiency: Another Inborn Error of Metabolism With Neonatal Onset

PEDIATRICS ◽  
1988 ◽  
Vol 82 (3) ◽  
pp. 521-521
Author(s):  
MASAFUMI MATSUO ◽  
EISAKU MAEDA ◽  
HAJIME NAKAMURA ◽  
KAYOKO SAIKI
Keyword(s):  
Inborn Error ◽  
Inborn Errors Of Metabolism ◽  
Early Recognition ◽  
Case Reports ◽  
Molybdenum Cofactor ◽  
Inborn Errors ◽  
Molybdenum Cofactor Deficiency ◽  
Neonatal Onset ◽  
Proper Diagnosis ◽  
Cofactor Deficiency

To the Editor.— We agree with Dr Burton1 that the practicing physician has the responsibility for the initial recognition of inborn errors of metabolism. Because we have experienced many cases of inborn errors of metabolism in neonates who might have died soon after birth without a proper diagnosis, we advocate that every neonatologist has the responsibility of recognizing these disorders. To promote the early recognition, we must know the initial signs or symptoms of neonatal cases of inborn errors of metabolism and we have collected neonatal case reports in the literatures during the last 5 years.

Microstructure and crystal symmetry of Pb2Sr2(Y/Ca)Cu3O9−δ

1990 ◽  
Vol 48 (4) ◽  
pp. 64-65
Author(s):  
Y. P. Lin ◽  
J. S. Xue ◽  
J. E. Greedan
Keyword(s):  
Electron Diffraction ◽  
High Temperature Superconductors ◽  
Carbon Film ◽  
Basic Structure ◽  
Crystal Symmetry ◽  
X Ray Diffraction ◽  
X Ray ◽  
Space Groups ◽  
New Family ◽  
Convergent Beam

A new family of high temperature superconductors based on Pb2Sr2YCu3O9−δ has recently been reported. One method of improving Tc has been to replace Y partially with Ca. Although the basic structure of this type of superconductors is known, the detailed structure is still unclear, and various space groups has been proposed. In our work, crystals of Pb2Sr2YCu3O9−δ with dimensions up to 1 × 1 × 0.25.mm and with Tc of 84 K have been grown and their superconducting properties described. The defects and crystal symmetry have been investigated using electron microscopy performed on crushed crystals supported on a holey carbon film.Electron diffraction confirmed x-ray diffraction results which showed that the crystals are primitive orthorhombic with a=0.5383, b=0.5423 and c=1.5765 nm. Convergent Beam Electron Diffraction (CBED) patterns for the and axes are shown in Figs. 1 and 2 respectively.

A new family of fluorescent calcium indicators designed to resist leakage or for measuring calcium near membranes

1994 ◽  
Vol 52 ◽  
pp. 168-169
Author(s):  
Martin Poenie ◽  
Akwasi Minta ◽  
Charles Vorndran
Keyword(s):  
Metal Binding ◽  
Acetoxymethyl Ester ◽  
Binding Properties ◽  
Fura 2 ◽  
New Family ◽  
Anion Transporter ◽  
Calcium Indicator ◽  
Calcium Indicators ◽  
High Calcium ◽  
Intracellular Compartmentalization

The use of fura-2 as an intracellular calcium indicator is complicated by problems of rapid dye leakage and intracellular compartmentalization which is due to a probenecid sensitive anion transporter. In addition there is increasing evidence for localized microdomains of high calcium signals which may not be faithfully reported by fura-2.We have developed a new family of fura-2 analogs aimed at addressing some of these problems. These new indicators are based on a modified bapta which can be readily derivatized to produce fura-2 analogs with a variety of new properties. The modifications do not affect the chromophore and have little impact on the spectral and metal binding properties of the indicator. One of these new derivatives known as FPE3 is a zwitterionic analog of fura-2 that can be loaded into cells as an acetoxymethyl ester and whose retention in cells is much improved. The improved retention of FPE3 is important for both cuvettebased measurements of cell suspensions and for calcium imaging.

Scanning tunneling microscopy and atomic force microscopy: New tools for biology

1989 ◽  
Vol 47 ◽  
pp. 778-779
Author(s):  
CE Bracker ◽  
P. K. Hansma
Keyword(s):  
Physical Property ◽  
Scanning Probe ◽  
Scanning Tunneling ◽  
Small Scale ◽  
Tunneling Microscopy ◽  
Force Microscopy ◽  
New Family ◽  
Small Probe ◽  
Atomic Force ◽  
Transmission Electron

A new family of scanning probe microscopes has emerged that is opening new horizons for investigating the fine structure of matter. The earliest and best known of these instruments is the scanning tunneling microscope (STM). First published in 1982, the STM earned the 1986 Nobel Prize in Physics for two of its inventors, G. Binnig and H. Rohrer. They shared the prize with E. Ruska for his work that had led to the development of the transmission electron microscope half a century earlier. It seems appropriate that the award embodied this particular blend of the old and the new because it demonstrated to the world a long overdue respect for the enormous contributions electron microscopy has made to the understanding of matter, and at the same time it signalled the dawn of a new age in microscopy. What we are seeing is a revolution in microscopy and a redefinition of the concept of a microscope.Several kinds of scanning probe microscopes now exist, and the number is increasing. What they share in common is a small probe that is scanned over the surface of a specimen and measures a physical property on a very small scale, at or near the surface. Scanning probes can measure temperature, magnetic fields, tunneling currents, voltage, force, and ion currents, among others.

Porphyrin-functionalized coordination star polymers and their potential applications in photodynamic therapy

2019 ◽  
Vol 10 (45) ◽  
pp. 6116-6121 ◽  
Author(s):  
Tan Ji ◽  
Lei Xia ◽  
Wei Zheng ◽  
Guang-Qiang Yin ◽  
Tao Yue ◽  
...  
Keyword(s):  
Photodynamic Therapy ◽  
Self Assembly ◽  
Star Polymers ◽  
New Family ◽  
Potential Applications

We present a new family of porphyrin-functionalized coordination star polymers prepared through combination of coordination-driven self-assembly and post-assembly polymerization. Their self-assembly behaviour in water and potential for photodynamic therapy were demonstrated.

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