Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “Protein Deficiency”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 271 records · Page 15Linked to original sources
PubMed1993

Mesenteric venous thrombosis in familial free protein S deficiency.

Protein S deficiency reportedly is associated with a high risk for thromboembolism, but mesenteric venous thrombosis is a rare manifestation of this hereditary disorder. We describe an additional case of mesenteric venous thrombosis in a 25-yr-old man with type I protein S deficiency. Total protein S level remained just below the normal range, but free protein S level was less than 10% of normal. Family study revealed that his father had had the same type of disorder. We have reviewed six well-documented cases of mesenteric venous thrombosis with protein S deficiency. It is particularly interesting that those with type I deficiency have had no previous thrombotic episodes (p < 0.05). Our patient was successfully treated with extended bowel resection, but unusual perioperative management was required. Because of extremely edematous mesentery and small bowel, we had to treat him with the abdomen wide open for 2 days between the primary and second laparotomies.

Adult↗
PubMed1984

Mucosal surface lesions in young protein-deficient pigs infected with Ascaris suum (Nematoda).

The morphology of the mucosal surface of samples from the small intestine of young protein-deficient pigs was examined by scanning electron microscopy. Three pigs had been infected with Ascaris suum for at least 58 days while 1 had remained uninfected; all pigs were housed and maintained under the same conditions, at the same time. The tissue samples collected at post mortem examination from pigs which contained A. suum showed varying degrees of villous atrophy and fusion. In addition, unusual small craters and as yet unidentified objects were observed at the surfaces of the enterocytes of Ascaris-infected pigs. These changes in mucosal morphology were not seen in the tissue taken from the uninfected pig. The possible association between the mucosal lesions and lactose maldigestion in young, protein-deficient pigs infected with A. suum is discussed.

Animals↗
PubMedDate not supplied

[Effect of certain essential amino acids excess on formation of aminoacyl-tRNA against a background of protein deficiency].

Optimal conditions are determined for the rat liver tRNA amino acylation with valine and methionine in norm, under protein deficiency and feeding of valine and methionine excess against a background of protein-free diet. It is shown that the level of the liver tRNA amino acylation with the protein deficiency is considerably higher than in the normal state. The valine and methionine excess against a background of protein-free diet inhibits the intensity of the valyl-tRNA and methionyl-tRNA formation. The cross experiments in a heterosystem show that this inhibition is due to changes in the activity or content of valyl- and methionyl-tRNA-synthetases in the rat liver.

Animals↗
PubMed1982

Effects of maternal protein deficiency on synaptic plasma membranes in offspring.

This study examined the influence of maternal protein deficiency during lactation on the accretion and synthesis of synaptic plasma membrane (SPM) proteins in developing offspring. It also examined the activity of Mg2+-dependent, ouabain-sensitive (Na+-K+)-ATPase in SPMs. The results of this study demonstrated that early postnatal undernutrition produces a significant deficit of SPM proteins. Despite this deficit, 10- to 20-day-old undernourished rats incorporated more [3H]-leucine into SPM proteins than age-matched control rats. The specific activity of (Na+-K+)-ATPase was decreased in SPMs from undernourished rats early in development, but was increased at 20 and 34 days of age. Interestingly, 20- and 34-day-old undernourished rats also had an increased proportion of [3H]-leucine-derived radioactivity in the band on SDS gels which corresponded to (Na+-K+)-ATPase. In summary, it appears that maternal deficiency of protein during lactation affects the synaptic plasma membranes in offspring quantitatively and qualitatively.

Animal Population Groups↗
PubMed1984

The use of a functional and immunologic assay for plasma protein C in the study of the heterogeneity of congenital protein C deficiency.

Protein C is a vitamin K dependent protein involved in blood coagulation. A congenital deficiency in protein C antigen - which inherits as an autosomal dominant disorder - has been reported to be associated with a high risk for thrombo-embolic disease at relatively young age. In the present paper we report on the development of a functional assay for plasma protein C. In this assay protein C is adsorbed to Al(OH)3, eluted and activated by thrombin, after which the concentration of the activated protein C is measured with a peptide substrate (S2366). Normal values for protein C activity and protein C antigen were determined in healthy volunteers and patients on stable oral anticoagulant treatment. Protein C activity and antigen levels were compared in 28 patients from 9 different pedigrees with both congenital protein C deficiency and thrombotic disease. Two types of protein C deficiency could be recognized: in type I the deficiency is due to the absence or reduced presence of protein C molecules, while in type II the deficiency is caused by the presence of an abnormal protein C molecule with strongly reduced functional activity.

Adult↗
PubMed1979

Effect of protein deficiency on the metabolism of glycoproteins and glycosaminoglycans in albino rat skin.

1. The total content of neutral sugars in skin of the weanling albino rats kept on the protein-deficient diet was increased by about 40%; this was mainly due to the increased concentration of galactose. The content of sialic acid was increased by about 20%. The collagen nitrogen was decreased significantly, with a concomitant increase of non-collagen nitrogen. At the same time, the content of sulphated glycosaminoglycans in skin was significantly decreased and that of non-sulphated glycosaminoglycans was increased. 2. Protein-deficient diet enhanced the activities of the protein-bound carbohydrate-degrading lysosomal hydrolases, viz. cathepsin D (EC 3.4.4.23), N-acetyl-beta-D-glucosaminidase (EC 3.2.1.30) and beta-D-glucuronidase (EC 3.2.1.31) both in liver and skin. The activity of liver hyaluronidase (EC 3.2.1.35) was also increased upon limitation of protein supply. 3. The changes observed in skin were accompanied by increased concentration of the protein-bound hexoses, hexosamines and sialic acids in serum, and of hexosamine and uronic acid in urine. The serum fucose remained unchanged.

Animals↗
PubMedDate not supplied

[Changes in the vessels of the kidneys in rats on a choline- and protein-deficient diet].

Prolonged (from 4 to 10 months) keeping of rats on a cholinoprotein- and protein-deficient diets produces an associated moderate damage to the wall on renal blood vessels and glomerulopathy. There take place dystrophic alterations in the elastic skeleton of the interlobular arteries in the form of elasticity loss and impairment of the tinctorial properties on the internal elastic membrane combined with vacuolization of the smooth muscle cells of the media.

Animals↗
PubMed1992

[Cerebral venous thrombosis and hereditary protein C deficiency].

Protein C together with its plasmatic cofactor protein S and antithrombin III probably represent the most important plasmatic inhibitor in coagulation. Protein C deficiency constitutes a high risk factor for venous thrombosis. Cerebral venous thrombosis is a manifestation which is scarcely referred to in protein C deficiency. The case of a 32 year old patient with protein C deficiency is presented. The patient was admitted for an endocraneal hypertension syndrome. CT and MR demonstrated multiple hemorrhagic cerebral infarctions. Arteriography confirmed vertebral venous thrombosis. Only six cases sufficiently documenting cerebral venous thrombosis due to protein C deficiency were found in the literature. In most cases coadjuvant factors exist predisposing thromboembolic disease. The present clinical case demonstrates the importance of considering protein C deficiency in the diagnosis of cerebral venous thrombosis in young adults.

Adult↗
PubMed1975

Exocrine pancreatic function in protein-deficient patas monkeys studied by means of a test meal and an indirect pancreatic function test.

Exocrine pancreatic secretion was studied in 9 patas monkeys before and during protein depletion, and in 4 of them also during recovery from protein depletion. Pancreatic function was estimated by measuring enzymatic activities in the duodenal contents after a test meal and by determination of urinary excretion of p-aminobenzoic acid (pancreatic function test) after oral ingestion of the chymotrypsin-labile peptide N-benzoyl-L-tyrosyl-p-aminobenzoic acid. The average serum albumin dropped by 34.8% to 2.6 g per 100 ml. Significant decrease of trypsin, lipase, amylase, and chymotrypsin was observed in the duodenal samples during protein deficiency. Urinary excretion of p-aminobenzoic acid was also reduced significantly. The two tests correlated well. In 3 of 4 animals, recovery of pancreatic function was noted after refeeding a full protein diet. Pancreatic atrophy was noted in 2 animals which died. The study shows that exocrine pancreatic secretion can be seriously impaired even at a moderate protein deficiency and may not be reversibly in all instances. Therefore, function tests have to be evaluated with caution when hypoproteinemia, i.e., hypoalbuminemia, is present.

Aminobenzoates↗
PubMed2003

Genetic rescue of chondrodysplasia and the perinatal lethal effect of cartilage link protein deficiency.

The targeted disruption of cartilage link protein gene (Crtl1) in homozygous mice resulted in a severe chondrodysplasia and perinatal lethality. This raised the question of whether the abnormalities seen in Crtl1 null mice are all caused by the absence of link protein in cartilage or whether the deficiency of the protein in other tissues and organs contributed to the phenotype. To address this question we have generated transgenic mice overexpressing cartilage link protein under the control of a cartilage-specific promoter, and then these transgenic mice were used for a genetic rescue of abnormalities in Crtl1 null mice. While the overexpression of cartilage link protein resulted in no abnormal phenotype, the cartilage-specific transgene expression of link protein could completely prevent the perinatal mortality of link protein-deficient mice and, depending on the level of the link protein expression, rescue skeletal abnormalities. Although link protein was originally isolated from cartilage, we found and determined Crtl1 transcripts and corresponding proteins in every organ tested from mouse embryos to aging animals. We also identified three additional members of the link protein family, all co-localized with hyaluronic acid-binding proteoglycans in the mouse genome. The ubiquitous presence of link protein suggests a general and systemic function of link protein in the organization of extracellular matrix in a number of tissues, possibly interacting with other proteoglycans, such as versican, brevican, and neurocan.

Animals↗
PubMed1978

Gastric mucosal barrier in the rats fed with protein deficient diet or treated with carbon tetrachloride.

The effects of protein deficient diet and liver damage on the gastric mucosal barrier were studied in 32 Sprague-Dawley rats. The animals were divided into 4 groups according to their dietary regimens, CCl4 induced liver damage and/or serotonin administration. Group 1 (control) was fed standard rat chow. Group II was fed a nonprotein diet for 2 weeks. Group III and IV were fed standard rat chow and given 1 ml of CCl4 per/Kg body weight intraperitoneally twice weekly for 12 weeks. Rats in Group IV were also injected with 20mg per/Kg of serotonin intraperitoneally 30 minutes prior to surgery. After pyloric ligation, an acid test solution composed of 100 mM HCl, 10mM NaCl, 5mM KCl and 5g per litre of PEG was instilled into the stomach and fluxes of Na +, K +, H + and Cl-ions across the gastric mucosa were measured. Compared to Group I, rats in Group II, III and IV demonstrated an increased net negative hydrogen flux (p less than 0.05). An increased net negative chloride flux (p less than 0.05) was also demonstrated in Group II and IV, but no increase was noted in Group III. There were no significant differences in fluxes of sodium and potassium ions. As mentioned above, a lack of protein in the diet, liver damage or both, renders the gastric mucosa of the rat more susceptible to injury. In the gastric mucosa of these rats, there have occurred more increase in the negative net fluxes of hydrogen and chloride ion, which may indicate the reduction of defence mechanism against a mucosal damage.

Animals↗
PubMed1999

Treatment of ataxia in isolated vitamin E deficiency caused by alpha-tocopherol transfer protein deficiency.

Dysfunction of the alpha-tocopherol transfer protein causes ataxia with isolated vitamin E deficiency. A 14-year-old male patient presented with ataxia and mental symptoms caused by a homozygous (552G-->A) alpha-tocopherol transfer protein mutation. After initiation of high-dosage alpha-tocopherol therapy, the organic mental syndrome disappeared and cognitive function improved rapidly. Neurologic recovery, however, was slow and incomplete.

Adolescent↗
PubMed1992

Suppression of muscle protein turnover and amino acid degradation by dietary protein deficiency.

To define the adaptations that conserve amino acids and muscle protein when dietary protein intake is inadequate, rats (60-70 g final wt) were fed a normal or protein-deficient (PD) diet (18 or 1% lactalbumin), and their muscles were studied in vitro. After 7 days on the PD diet, both protein degradation and synthesis fell 30-40% in skeletal muscles and atria. This fall in proteolysis did not result from reduced amino acid supply to the muscle and preceded any clear decrease in plasma amino acids. Oxidation of branched-chain amino acids, glutamine and alanine synthesis, and uptake of alpha-aminoisobutyrate also fell by 30-50% in muscles and adipose tissue of PD rats. After 1 day on the PD diet, muscle protein synthesis and amino acid uptake decreased by 25-40%, and after 3 days proteolysis and leucine oxidation fell 30-45%. Upon refeeding with the normal diet, protein synthesis also rose more rapidly (+30% by 1 day) than proteolysis, which increased significantly after 3 days (+60%). These different time courses suggest distinct endocrine signals for these responses. The high rate of protein synthesis and low rate of proteolysis during the first 3 days of refeeding a normal diet to PD rats contributes to the rapid weight gain ("catch-up growth") of such animals.

Alanine↗
PubMed2002

Clara cell secretory protein deficiency alters clara cell secretory apparatus and the protein composition of airway lining fluid.

Clara cells represent the predominant secretory cell within distal conducting airways of mammals and exhibit functional alterations with chronic lung disease. We previously demonstrated that Clara cell secretory protein (CCSP) deficiency results in enhanced susceptibility to environmental agents. The present study was undertaken to define changes in Clara cell secretory function associated with CCSP deficiency in knockout mice. Comparative morphometry of Clara cell ultrastructure revealed dramatic alterations in secretory apparatus between wild-type (WT) and CCSP knockout (CCSP-/-) mice. Secretory granules, which occupy greater than 2% of Clara cell cytoplasmic volume in WT mice, were completely absent among Clara cells of CCSP-/- mice. Moreover, Clara cells of CCSP-/- mice exhibited a > 95% reduction in rough endoplasmic reticulum and alterations to Golgi apparatus, relative to WT controls. Ultrastructural perturbations to Clara cells were associated with altered protein composition of airway lining fluid as revealed by two-dimensional gel analysis of bronchoalveolar lavage proteins, but were not associated with altered abundance or secretion of CC26, another Clara cell secretory protein. We conclude that CCSP is required for the appearance of Clara cell secretory granules and that functional changes to Clara cells that result from CCSP deficiency lead to alterations in the composition of epithelial lining fluid.

Animals↗