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Biomedical subjects

P Perutelli

Publications and source records attributed to P Perutelli.

At least 37 records · Page 2Linked to original sources
PubMed1989

A simple, rapid method for screening hybridomas producing monoclonal antibodies to platelet proteins.

Several parameters influence the outcome of somatic cell fusions based on the Köhler and Milstein technology, and a number of steps are of critical importance, including the screening strategy. The procedure chosen, appropriate for the type of antibody required, should be rapid and sensitive, in order to clone the relevant hybrids as quickly as possible. A simple and quick dot blot-based method is reported, suitable for screening hybridoma culture supernatants in order to identify clones producing monoclonal antibodies to platelet constituents.

Animals↗
PubMed1978

Lymphoid cell surface markers in acute lymphocytic leukaemia.

Peripheral blood lymphoid cells of 29 patients with acute lymphocytic leukaemia (ALL) at onset were studied for characterization of B and T membrane markers and phytohaemagglutinin responsiveness. 24 cases (83%) were classified as "null" cell ALL and 5 (17%) as T-cell ALL. No relationship could be found between cytological presentation and immunological classification. Moreover, no correlation has been demonstrated between clinical-immunological parameters and prognosis, indicating that in our series of patients, assessment of cell size and surface markers were not a reliable predictor of prognosis.

Antineoplastic Agents↗
PubMed1977

Immunological evaluation of 15 children with non-Hodgkin lymphoma.

In the present study, 15 children with non-Hodgkin lymphoma have been immunologically evaluated by the following parameters on peripheral blood (PB) lymphocytes: phytohemagglutinin responsiveness (PHA-r); non-immune rosette formation with sheep red blood cells (RF-L) as T-cell marker; presence of surface immunoglobulins (sIg-L) as B-cell marker; serum immunoglobulins levels (IgA, IgM, IgG). Our patients (pts) have been divided in two groups: the first one includes 10 children without PB involvement; the second one includes 5 pts with bone-marrow and PB invasion. From our data it appears that: 1) the majority of pts of the first group presented normal values of membrane markers; PHA-r was impaired in 4/8 pts; 2) in pts with PB invasion absolute number of B and "null" cells was always abnormal and PHA-r altered; 3) in the second group of pts, a "null" cell origin can be suggested by the high percentage and absolute number of cells without surface markers. In our opinion, the high incidence of "null" cells represents the most relevant question: whether they are cells deprived of specific markers, or endowed with markers not identifiable by our current techniques, remains to be established.

Adolescent↗
PubMed1976

Aspecific transfer factor in children with Hodgkin's disease.

Six children suffering from Hodgkin's disease (HD), in different stages and free from chemo- and radiotherapy from at least four weeks, were treated with transfer factor (TF). PPD skin test, PHA-responsiveness, E-rosettes, B-lymphocytes were checked before TF therapy, after 6 and 9 TF doses and compared to the data at the onset of the disease. Two children with HD who did not receive TF, were examined as controls. PPD skin tests, PHA-responsiveness, E-rosettes did not ameliorate following TF treatment, while it has been noticed an increase in B-lymphocytes, both in percentage and absolute number. The Authors conclude that TF might have induced a slight B lymphoproliferation.

B-Lymphocytes↗
PubMedDate not supplied

von Willebrand factor: biological function and molecular defects.

The human von Willebrand factor (vWF) plays a pivotal role in the mechanisms of blood clotting and platelet thrombus formation; it also binds and stabilizes factor VIII procoagulant protein. The biological functions of vWF are dependent on distinct molecular domains responsible for the specificity and affinity for ligands. The multimeric structure of vWF provides an array of binding sites that allow multivalent interactions, thus supporting the formation of stable platelet aggregates at the site of vascular injury, particularly under flow conditions characterized by high shear stress. Quantitative and qualitative abnormalities of vWF cause the most common congenital bleeding disorder in humans, the von Willebrand disease (vWD). This review will provide an update on the recent advances toward the elucidation of structure-function relationships and the detection of molecular defects leading to vWD and will highlight the revised classification of vWD.

Heparin↗
PubMedDate not supplied

The human platelet membrane glycoprotein IIb/IIIa complex: a multi functional adhesion receptor.

The glycoprotein GPIIb/IIIa complex is a major constituent of the platelet membrane; it plays an important role in platelet adhesion and aggregation. The complex is a member of the integrin superfamily. Integrins are related membrane receptors which mediate the adhesive interactions of a variety of cells; they specifically recognize the arginine-glycine-aspartic acid (RGD) sequence present in several adhesive proteins. The GPIIb/IIIa complex of activated platelets can bind fibrinogen, von Willebrand factor, fibronectin, vitronectin and thrombospondin. Platelets are activated by a variety of signals including extracellular matrix molecules and soluble factors; upon platelet activation the complex undergoes a conformational change, thus permitting the macromolecular ligands access to their binding sites. In turn, fibrinogen binding results in a receptor modification and neoantigens exposure; such events may participate in signal transduction. The adhesive proteins compete reciprocally for binding to GPIIb/IIIa, and the complex binds to different domains of them, thus creating multiple interactions with the ligands.

Amino Acid Sequence↗
PubMedDate not supplied

Biochemical and molecular basis of Glanzmann's thrombasthenia.

Glanzmann's thrombasthenia is a rare autosomal recessive bleeding disorder characterized by a quantitative deficiency or a functional abnormality of the major platelet membrane integrin receptor: the glycoprotein (GP) IIb/IIIa complex. The GPIIb/IIIa complex functions as a platelet receptor for fibrinogen, von Willebrand factor, fibronectin and vitronectin; therefore it plays an important role in platelet adhesion and aggregation. Thrombasthenic platelets are severely deficient in GPIIb/IIIa content or function, and fail to aggregate and form the hemostatic plug at the site of vessel injury. On the other hand, heterozygous subjects (having about half the number of normal GPIIb/IIIa complexes) do not show bleeding problems. It has been demonstrated that a molecular defect affecting one of the two GP coding genes is sufficient to determine a contemporary deficit of both GPIIb and GPIIIa, and hence the thrombasthenic phenotype. Up to now, few molecular abnormalities giving rise to Glanzmann's thrombasthenia have been characterized. Large rearrangements within the GPIIb or GPIIIa coding genes appear to be unusual, whereas small modifications in the nucleotide sequence of the coding regions occur with higher frequency.

Chromosome Deletion↗
PubMedDate not supplied

Platelet glycoprotein Ib polymorphism in the Italian population.

The glycoprotein Ib (GP Ib) is the major sialoglycoprotein on the platelet membrane and plays an important role in primary hemostasis. It was demonstrated that GP Ib is polymorphic: four different species of GP Ib (designated A, B, C and D) were observed, having molecular weights of 168, 162, 159 and 153 kiloDaltons, respectively. The polymorphism was first studied in the Japanese population and, subsequently, in Americans; a significant difference between the phenotype frequencies in the two populations was found. The authors report about GP Ib polymorphism patterns in the Italian population. GP Ib from normal subjects was analyzed by means of platelet protein SDS-PAGE followed by Western blotting; GP Ib species immobilized onto nitrocellulose filters were coupled with wheat germ agglutinin and stained using immunoperoxidase. Despite some differences in phenotype distribution, statistical analysis did not show differences between Italian and U.S. Caucasian phenotype frequencies: this is attributable to rather similar gene frequencies in the two populations. In our platelet samples we only observed three GP Ib types, precisely the B, C and D types; therefore we can confirm the rarity of the A type in Caucasians, while it was well represented in the Japanese population.

Alleles↗
PubMedDate not supplied

[Epidemiologic research for thalassemia and hemoglobinopathy traits in the territory of a local health unit in Liguria].

The authors investigated the incidence of thalassemia traits and hemoglobinopathies in western Liguria, where up to 70% of people comes from other italian regions, particularly from the South. The authors screened 442 primary school pupils in Albenga and Andora (Savona). Laboratory investigations permitted to detect 19 thalassemia trait carrier subjects (4.30% of the total examined): 12 of them were diagnosed heterozygous for beta-thalassemia, 6 for alpha-thalassemia, and 1 for Hb S. Authors would underline that more than half of the screening positive subjects resulted carrier of beta-thalassemia or Hb S trait, both potentially able to give origin to severe diseases: homozygous beta-thalassemia, sickle cell anemia, and beta-thalassemia/Hb S double heterozygosity.

Anemia, Sickle Cell↗