scholarly journals The molecular mechanism of the inherited phosphofructokinase deficiency associated with hemolysis and myopathy

Blood ◽  
1980 ◽  
Vol 55 (4) ◽  
pp. 629-635
Author(s):  
S Vora ◽  
L Corash ◽  
WK Engel ◽  
S Durham ◽  
C Seaman ◽  
...  
Keyword(s):  
Molecular Basis ◽  
Direct Evidence ◽  
The Other ◽  
Heterogeneous Mixture ◽  
Muscle Type ◽  
Crossover Point ◽  
Normal Human ◽  
Phosphofructokinase Deficiency ◽  
2,3 Dpg ◽  
Chromatographic Profiles

Normal human erythrocyte phosphofructokinase (ATP:c D-fructose-6, P-1- phosphotransferase, EC 2.7.1.11; PFK) has recently been shown to consist of a heterogeneous mixture of five tetrameric isozymes: M4, M3L, M2L2, ML3, and L4 (M, muscle type; L, liver type). In the light of these findings, we have investigated the molecular basis of the inherited erythrocyte PFK deficiency associated with myopathy and hemolysis (Tarui disease). The propositus, a 31-yr-old male, suffered from muscle weakness and myoglobinuria on exertion. He showed mild erythrocytosis despite laboratory evidence of hemolysis. In his erythrocytes a metabolic crossover point was found at the level of PFK; 2,3-diphosphoglycerate (2,3-DPG) was also significantly reduced. The PFK from the patient's erythrocytes consisted exclusively of the L4 isozyme, and there was a complete absence of the other four. The leukocyte and platelet PFKs from the patient showed normal activities, chromatographic profiles, and precipitation with anti-M4 antibody. These studies provide direct evidence that in Tarui disease the M-type subunits are absent; but the liver- and platelet-type subunits of PFK are unaffected. The paradox of mild erythrocytosis despite hemolysis reflects the decreased production of 2,3-DPG.

scholarly journals The molecular mechanism of the inherited phosphofructokinase deficiency associated with hemolysis and myopathy

Blood ◽  
1980 ◽  
Vol 55 (4) ◽  
pp. 629-635 ◽  
Author(s):  
S Vora ◽  
L Corash ◽  
WK Engel ◽  
S Durham ◽  
C Seaman ◽  
...  
Keyword(s):  
Molecular Basis ◽  
Direct Evidence ◽  
The Other ◽  
Heterogeneous Mixture ◽  
Muscle Type ◽  
Crossover Point ◽  
Normal Human ◽  
Phosphofructokinase Deficiency ◽  
2,3 Dpg ◽  
Chromatographic Profiles

Abstract Normal human erythrocyte phosphofructokinase (ATP:c D-fructose-6, P-1- phosphotransferase, EC 2.7.1.11; PFK) has recently been shown to consist of a heterogeneous mixture of five tetrameric isozymes: M4, M3L, M2L2, ML3, and L4 (M, muscle type; L, liver type). In the light of these findings, we have investigated the molecular basis of the inherited erythrocyte PFK deficiency associated with myopathy and hemolysis (Tarui disease). The propositus, a 31-yr-old male, suffered from muscle weakness and myoglobinuria on exertion. He showed mild erythrocytosis despite laboratory evidence of hemolysis. In his erythrocytes a metabolic crossover point was found at the level of PFK; 2,3-diphosphoglycerate (2,3-DPG) was also significantly reduced. The PFK from the patient's erythrocytes consisted exclusively of the L4 isozyme, and there was a complete absence of the other four. The leukocyte and platelet PFKs from the patient showed normal activities, chromatographic profiles, and precipitation with anti-M4 antibody. These studies provide direct evidence that in Tarui disease the M-type subunits are absent; but the liver- and platelet-type subunits of PFK are unaffected. The paradox of mild erythrocytosis despite hemolysis reflects the decreased production of 2,3-DPG.

scholarly journals Molecular Basis of Canine Muscle Type Phosphofructokinase Deficiency

1996 ◽  
Vol 271 (33) ◽  
pp. 20070-20074 ◽  
Author(s):  
Bruce F. Smith ◽  
Hansell Stedman ◽  
Yashoda Rajpurohit ◽  
Paula S. Henthorn ◽  
John H. Wolfe ◽  
...  
Keyword(s):  
Molecular Basis ◽  
Muscle Type ◽  
Phosphofructokinase Deficiency

IMMUNOLOGICAL REACTIVITY OF INSULIN IN HUMAN SERUM

1966 ◽  
Vol 53 (4) ◽  
pp. 673-680 ◽  
Author(s):  
Torsten Deckert ◽  
Kai R. Jorgensen
Keyword(s):  
Normal Subjects ◽  
The Other ◽  
Human Pancreas ◽  
Porcine Pancreas ◽  
Crystalline Insulin ◽  
Endogenous Insulin ◽  
Significant Difference ◽  
Human Sera ◽  
Normal Human ◽  
The One

ABSTRACT The purpose of this study was to investigate whether a difference could be demonstrated between crystalline insulin extracted from normal human pancreas, and crystalline insulin extracted from bovine and porcine pancreas. Using Hales & Randle's (1963) immunoassay no immunological differences could be demonstrated between human and pig insulin. On the other hand, a significant difference was found, between pig and ox insulin. An attempt was also made to determine whether an immunological difference could be demonstrated between crystalline pig insulin and crystalline human insulin from non diabetic subjects on the one hand and endogenous, circulating insulin from normal subjects, obese subjects and diabetic subjects on the other. No such difference was found. From these experiments it is concluded that endogenous insulin in normal, obese and diabetic human sera is immunologically identical with human, crystalline insulin from non diabetic subjects and crystalline pig insulin.

The Origins of Domestic Sheep and Goats: a Reconsideration in the Light of the Fossil Evidence

1969 ◽  
Vol 34 ◽  
pp. 368-384 ◽  
Author(s):  
Sebastian Payne
Keyword(s):  
Direct Evidence ◽  
Present Situation ◽  
Wild Relatives ◽  
The Other ◽  
Domestic Sheep ◽  
Sheep And Goats ◽  
Animal Domestication ◽  
The Past ◽  
The One ◽  
And Control

In recent discussions of the origins and process of animal domestication (Reed, 1961, Zeuner, 1963), both authors rely on two kinds of evidence: on the one hand, the present distributions and characteristics of the different breeds of whatever animal is being discussed, together with its feral and wild relatives, and, on the other hand, the past record, given by literary and pictorial sources and the bones from archaeological and geological sites. Increased recognition of the limitations of the past record, whether in the accuracy of the information it appears to give (as in the case of pictorial sources), or in the certainty of the deductions we are at present capable of drawing from it (this applies especially to the osteological record), has led these authors to argue mainly from the present situation, using the past record to confirm or amplify the existing picture.Arguing from the present, many hypotheses about the origins and process of domestication are available. The only test we have, when attempting to choose between these, lies in the direct evidence of the past record. The past record, it is freely admitted, is very fragmentary: the information provided by the present situation is more exact, ranges over a much wider field, and is more open to test and control. Nevertheless, the past record, however imperfect it is, is the only direct evidence we have about the process of domestication.

Abnormal vascular function and morphology in preeclampsia: a study of isolated resistance vessels

1985 ◽  
Vol 69 (4) ◽  
pp. 477-482 ◽  
Author(s):  
C. Aalkjaer ◽  
H. Danielsen ◽  
P. Johannesen ◽  
E. B. Pedersen ◽  
A. Rasmussen ◽  
...  
Keyword(s):  
Angiotensin Ii ◽  
Pregnant Women ◽  
Vascular Function ◽  
Direct Evidence ◽  
The Other ◽  
Vascular Changes ◽  
Direct Information ◽  
Vascular Abnormalities ◽  
Media Thickness ◽  
Resistance Vessels

1. In order to obtain direct information about vascular changes associated with pre-eclampsia, the morphological and functional characteristics of isolated omental resistance vessels from 11 women with pre-eclampsia, 10 normotensive pregnant women and eight normotensive nonpregnant women were determined. 2. In vessels from the women with preeclampsia, the ratio of media thickness to lumen diameter was increased, compared with that in vessels from the other two groups. 3. The vessels from the women with preeclampsia had an increased responsiveness to angiotensin II and a decreased rate of relaxation, but only when compared with the vessels from the normotensive pregnant women. However, no difference in responsiveness to noradrenaline was found between any of the groups. 4. The angiotensin II responsiveness of the vessels from the women with pre-eclampsia and from the non-pregnant women were similar, suggesting that pre-eclampsia is associated with an absence of the change in vascular function which normally occurs during pregnancy. 5. The study provides direct evidence for an involvement of vascular abnormalities in the pathogenesis of pre-eclampsia.

Photoreceptor coupling in turtle retina

1998 ◽  
Vol 15 (4) ◽  
pp. 755-764 ◽  
Author(s):  
M.L. FIRSOV ◽  
D.G. GREEN
Keyword(s):  
Receptive Field ◽  
Gap Junctions ◽  
Direct Evidence ◽  
Physiological Responses ◽  
The Other ◽  
Morphological Data ◽  
Chelydra Serpentina ◽  
Intracellular Injection ◽  
Coupled Cells ◽  
High Degree

Photoreceptors in the isolated turtle retina of two species of turtle, Chelydra serpentina and Pseudemus scripta elegans, were penetrated with double-barrel electrodes. Physiological responses were recorded through one barrel and Neurobiotin tracer was injected from the other. Intracellular injection of Neurobiotin revealed patterns of tracer-coupled photoreceptors. Both the patterns of tracer coupling and the electrophysiology suggest a high degree of specificity of connections. Rods seem to be coupled only to rods and green and red cones seem to be coupled to cones of the same spectral type. Receptive-field profiles, measured with a thin, sharply focused slit of light, often had well-defined peaks and troughs in sensitivity. We have taken advantage of this observation and used the position of a peak in sensitivity to locate the position on the retina of a coupled cell. In one rod, it was possible to correlate physiological and morphological data and to show that the peaks in the physiological receptive field occurred at positions on the retina where there were dye-coupled cells. This provides direct evidence that gap junctions produce the physiological coupling between rods.

The Stoic Ontology of Geometrical Limits

Phronesis ◽  
2009 ◽  
Vol 54 (4-5) ◽  
pp. 371-389 ◽  
Author(s):  
Anna Eunyoung Ju
Keyword(s):  
Direct Evidence ◽  
The Other ◽  
Specific Topic ◽  
Textual Evidence ◽  
The One ◽  
Metaphysical Status

AbstractScholars have long recognised the interest of the Stoics' thought on geometrical limits, both as a specific topic in their physics and within the context of the school's ontological taxonomy. Unfortunately, insufficient textual evidence remains for us to reconstruct their discussion fully. The sources we do have on Stoic geometrical themes are highly polemical, tending to reveal a disagreement as to whether limit is to be understood as a mere concept, as a body or as an incorporeal. In my view, this disagreement held among the historical Stoics, rather than simply reflecting a doxographical divergence in transmission. This apparently Stoic disagreement has generated extensive debate, in which there is still no consensus as to a standard Stoic doctrine of limit. The evidence is thin, and little of it refers in detail to specific texts, especially from the school's founders. But in its overall features the evidence suggests that Posidonius and Cleomedes differed from their Stoic precursors on this topic. There are also grounds for believing that some degree of disagreement obtained between the early Stoics over the metaphysical status of shape. Assuming the Stoics did so disagree, the principal question in the scholarship on Stoic ontology is whether there were actually positions that might be called "standard" within Stoicism on the topic of limit. In attempting to answer this question, my discussion initially sets out to illuminate certain features of early Stoic thinking about limit, and then takes stock of the views offered by late Stoics, notably Posidonius and Cleomedes. Attention to Stoic arguments suggests that the school's founders developed two accounts of shape: on the one hand, as a thought-construct, and, on the other, as a body. In an attempt to resolve the crux bequeathed to them, the school's successors suggested that limits are incorporeal. While the authorship of this last notion cannot be securely identified on account of the absence of direct evidence, it may be traced back to Posidonius, and it went on to have subsequent influence on Stoic thinking, namely in Cleomedes' astronomy.

scholarly journals Secretion of β-N-acetylglucosaminidase isoenzymes by normal human fibroblasts

1978 ◽  
Vol 173 (2) ◽  
pp. 433-439 ◽  
Author(s):  
P Willcox
Keyword(s):  
Lysosomal Enzyme ◽  
Human Fibroblast ◽  
Lysosomal Enzymes ◽  
Culture Medium ◽  
Human Fibroblasts ◽  
The Other ◽  
Normal Human Fibroblast ◽  
Acid Hydrolases ◽  
Human Fibroblast Cultures ◽  
Normal Human

1. Secretion of the lysosomal enzyme beta-N-acetylglucosaminidase (EC 3.2.1.30) by normal human fibroblast cultures was linear with respect to time up to 96h. 2. Two forms of the A isoenzyme of beta-N-acetylglucosaminidase were found in the culture medium. One form was similar to the isoenzyme found in other extracellular fluids, such as plasma and tears, the other resembled the intracellular (lysosomal) enzyme. The presence of the two isoenzymes in the culture medium appears to reflect two distinct secretory processes. 3. It is suggested that plasma acid hydrolases may be destined for incorporation into lysosomes in a manner analogous to that described for the packaging of lysosomal enzymes by fibroblasts.

Pointing, Rainbows, and the Archaeology of Mind

Anthropos ◽  
2021 ◽  
Vol 116 (1) ◽  
pp. 145-162
Author(s):  
Robert Blust
Keyword(s):  
Index Finger ◽  
The Other ◽  
Geographical Region ◽  
Cultural Backgrounds ◽  
Human Interactions ◽  
Normal Human ◽  
Other World

For over a century anthropologists and folklorists have sporadically recorded a belief that one should not point at a rainbow, lest the offending finger become permanently bent, rot, be supernaturally severed, fall off, etc. In each case the belief was reported for a particular geographical region without apparent awareness of its presence elsewhere, and in no case was an explanation for this curious idea proposed. This paper documents what is called the “Rainbow Taboo” as a global phenomenon, found among peoples of quite varied cultural backgrounds, and it argues that the universality of the belief is a product of the interaction of two independent cognitive elements: an apparently innate sense that the rainbow is associated with the “other world,” and, secondly, a similar sense that pointing with the index finger is aggressive, and should not be used either in normal human interactions or more particularly against the supernatural.

Studies on the Nature of the Plasma Erythropoietic Factor(s)

PEDIATRICS ◽  
1958 ◽  
Vol 21 (4) ◽  
pp. 581-581
Keyword(s):  
The Other ◽  
Normal Human Plasma ◽  
Erythropoietic Activity ◽  
Cellular Division ◽  
Humoral Factors ◽  
Hemoglobin Variability ◽  
Heat Stable ◽  
Hemopoietic Tissue ◽  
Blood Formation ◽  
Normal Human

In recent years the existence of a humoral factor or factors which stimulate erythropoietic activity has been demonstrated. The present paper reports further studies on the nature of this erythropoietic factor or factors. The author's studies indicate that there are at least two humoral erythropoietic facts. One is heat stable and appears to exert its effects upon cellular division of erythrocyte precursers in the marrow. The other factor is relatively thermolabile and exerts its effect through augmentation of incorporation of iron into hemoglobin. Variability in experimental results which have been reported in studies of the humoral erythropoietic factor may, in the authors' opinion, be due to differences in the material being studied (in content of these factors depending upon how the material being tested was prepared). These humoral factors are present in normal human plasma which suggests that they are involved in the maintenance of normal blood formation. Increased amounts of these factors in plasma in some anemias may be the result of local tissue hypoxia. In polycythemia vera the humoral factors may be of pathogenetic importance. The factors do not appear to be formed in hemopoietic tissue; the kidney has been suggested as a possible locus of formation.

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