Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0032463 (polycythemia vera)
 3,374 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In order to evaluate the role of proteolysis in acquired von Willebrand's disease (vWD) associated with the myeloproliferative syndrome, we have determined the relative quantity of von Willebrand factor (vWF) fragments as compared with the intact 225 kDa subunit in four patients. The plasma vWF of each individual lacked large multimers; each had a prolonged bleeding time; and both platelet and leukocyte counts were elevated. Plasma was obtained from blood drawn into 1 mmol/L leupeptin, 6 mmol/L N-ethylmaleimide, and 5 mmol/L EDTA to prevent in vitro proteolysis. vWF was isolated from plasma by immunoadsorbent chromatography, reduced, subjected to SDS-5% polyacrylamide gel electrophoresis, and immunoblotted with a mixture of 55 anti-vWF monoclonal antibodies. In three patients with essential thrombocytosis (ET) the 176 and 140 kDa fragments were increased in proportion to the intact 225 kDa subunit indicating increased proteolysis. Treatment of one ET patient with CCNU (Lomustine) decreased the platelet count and, to a lesser extent, the white blood cell count. This was associated with a correction of the bleeding time, a partial correction of the multimeric abnormality, and a lessening of vWF cleavage. In a patient with polycythemia rubra vera (PRV) the proportion of the 176 kDa fragment was increased to the upper limit of normal but there was no definite evidence of increased proteolysis. These studies provide evidence that proteolysis plays a role in the acquired von Willebrand's disease associated with the myeloproliferative syndrome. However, other mechanisms must also be considered.
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PMID:Subunit composition of plasma von Willebrand factor in patients with the myeloproliferative syndrome. 353 24

Neutrophil granule subsets and dynamics were studied in 4 patients with polycythemia vera/myelofibrosis and 2 patients with chronic myelogenous leukemia. Alkaline phosphatase, a marker for the membrane of secretory vesicles (the most readily mobilizable pool of intracellular membranes in neutrophils) was highly elevated in the PV/MF patients and significantly reduced in the CML patients. In spite of this, the amount of secretory vesicles was normal as judged by the content of albumin, and of the membrane protein cytochrome b-245 and CD11b, both partially localized in secretory vesicles. Gelatinase granules were present in all patients. The azurophil granules were lighter than normal in both CML patients. SDS-PAGE protein profiles indicated absence of defensins from azurophil granules from 1 CML patient. In addition, a 41-42 kD doublet protein band was absent from 2 PV and 1 CML patient, and reduced in the other CML patient. No difference in mobilization of granules was observed between patient neutrophils and control neutrophils. Also, stimulation with 10(-8) mol/l N-formyl-methionyl-leucyl-phenylalanine induced normal increases in intracellular Ca2+ in patient neutrophils. These results indicate that stimulus-response coupling leading to granule exocytosis is intact in neutrophils from patients with myeloproliferative disorders.
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PMID:Mobilization of granules in neutrophils from patients with myeloproliferative disorders. 838 6

The envelope glycoprotein gD gene of pseudorabies virus Ea strain was cloned via PCR technique. Sequence analysis displayed 98% nucleotide sequence homology and 97% deduced amino acid sequence homology between our cloned gD gene and PRV Rice strain gD gene. The recombinant transfer plasmid pSX35A-gD was obtained by inserting D gene into the baculovirus transfer vector pSX35A with whole-phase promoter cassette, then transfected insect cell Hi5 with linearized AcMNPV-OCC- virus DNA, and formed recombinant baculoviruses AcMNPV-OCC(+)-gD by homologous recombination in insect cell. Recombinant baculoviruses infected insect cell Hi5 after being purified by plaque assay. Both SDS-PAGE and Western-blotting showed glycoprotein gD with a molecular weight of about 47 kD was expressed specifically, product was about 6.2% of total cellular protein, and expressed gD was of immunogenicity.
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PMID:[Cloning and expression of the envelope glycoprotein gD gene of pseudorabies virus EA strain]. 1254 87

Pseudorabies virus glycoprotein E (PRV gE) has been recognized as a suitable diagnostic antigen for pseudorabies. In order to produce gE antigen in large quantities and at low cost, a gene fragment encoding PRV gE epitopes was expressed in Pichia pastoris expression system. SDS-PAGE and Western blotting revealed that the expression product was two recombinant proteins, approximately 38 and 32 kDa, in the culture supernatant of P. pastoris integrant 72 h after induction. Protein concentration assay showed the expression product amounted to 106.7 mg/l, accounting for 66.67% of total culture supernatant proteins. An indirect PRV gE-ELISA was then established by using the recombinant expression product as a coating antigen. Cross-reactivity assay showed that this antigen was PRV specific. Reproducibility experiment displayed good consistency. Comparison of detection results of 348 field serum samples between PRV gE-ELISA and a commercially available PRV diagnostic kit showed there was no significant difference between these two methods (P > 0.05).
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PMID:Expression of pseudorabies virus gE epitopes in Pichia pastoris and its utilization in an indirect PRV gE-ELISA. 1462 49

During lytic infection, the virion host shutoff (vhs) protein of alphaherpesviruses causes the degradation of mRNAs nonspecifically. In this work, we cloned the vhs gene (UL41 open reading frame) of pseudorabies virus (PRV; TNL strain) by PCR, and its nucleotide sequences were determined. The PCR product of vhs gene was subcloned into the prokaryotic pET32b expression vector, and production of the recombinant vhs protein was examined by SDS-PAGE. Result of Western blotting demonstrated that our recombinant vhs protein reacted with antiserum against a synthetic peptide of 17 amino acids of the vhs protein. After purification with nickel-chelate affinity chromatography, the purified recombinant vhs protein exhibited in vitro ribonuclease activity as expected. We further cloned the vhs gene into eukaryotic expression vectors and investigated the intracellular function of vhs protein by DNA transfection. By transient transfection and CAT assay, we found the CAT activity was reduced in the presence of vhs, indicating that degradation of mRNA of the CAT gene was caused by the vhs. Furthermore, our results showed that the plaque formation of pseudorabies virus was blocked by exogenous vhs. Taken together, we have cloned the vhs gene of pseudorabies virus (TNL strain) and conducted functional analysis of the recombinant vhs protein in vitro as well as in vivo.
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PMID:Functional analysis of virion host shutoff protein of pseudorabies virus. 1520 26