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Query: UNIPROT:P15088 (mast cell)
 14,925 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Mastocytosis is a common feature around solid tumors. Due to mast cell (MC) degranulation, heparin and other chemical mediators are released to surrounding tissues. The aim of this paper is to investigate the role of heparin and chemically modified heparins, on a murine mammary adenocarcinoma cell line adhesion properties, and the relationship with the presence of heparin binding sites in tumor cells. We show that heparin increases tumor cell adhesion in a dose-dependent manner. When the number of heparin binding sites was regulated, by culturing the cells with different FCS concentration for 24 hours, a correlation between binding capacity and heparin effect on cell adhesion was observed. The increment on cell adhesion by heparin was lower on cells with less heparin binding sites. Moreover, only heparin and a chemically modified heparin (partially N-desulfated N-acetylated), which bound to heparin-receptor, retained the ability to stimulate cell adhesion, while other modified heparins lost both effects. The increase in cell adhesion was observed on plastic dishes, albumin, as well as on fibronectin pre-coated ones suggesting that heparin effect is substratum independent. Our results show a direct relation between heparin binding to specific cell receptors and increase in cell attachment.
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PMID:Heparin increases the adhesion of murine mammary adenocarcinoma cells (LM3). Correlation with the presence of heparin receptors on cell surface. 1617 72

Phosphoinositide-3-kinases (PI3Ks) are a family of lipid kinases essential in a variety of physiological reactions. A series of gene-targeted mice lacking different PI3Ks and related molecules has enabled us to understand their in vivo roles, particularly those of class IA members. Studies on knockout mice lacking class IA PI3Ks and knock-in mice expressing mutant forms of enzymes have revealed the importance of this class of PI3Ks in mast cell development in the gastrointestinal tract. Here we studied the role of the p85a regulatory subunit, the most abundantly expressed regulatory subunit of class IA PI3Ks, using p85a knockout mice. Development of mast cells in the gastrointestinal tract but not in the skin was severely impaired in mice lacking the p85a regulatory subunit. Stem cell factor (SCF)-mediated signalling functions including proliferative response and chemotactic activities were both impaired in p85a knockout mast cells, likely due to the mast cell deficiency. Mastocytosis upon Strongyloides veneZuelensis infection was also impaired in p85alpha knockout mice. Reconstitution with Th2-conditioned but not untreated bone marrow-derived mast cells (BMMCs) restored anti-bacterial immunity, indicating the importance of Th2 response in addition to the recruitment of mast cells in the control of nematode infection.
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PMID:The role of phosphoinositide-3-kinase in mast cell homing to the gastrointestinal tract. 1660 33

Mastocytosis refers to a rare collection of disorders, both cutaneous and systemic, that are characterized by increased numbers of mast cells. Depending on the extent of the disease, these disorders may present with symptoms resulting from mast cell degranulation including flushing, diarrhea, vomiting, cramping, syncope, or anaphylaxis. In pediatric patients, cutaneous involvement is most prevalent in the form of urticaria pigmentosa, which is typically asymptomatic or minimally so with resolution by adolescence. In this case report and review of literature, we review a case of a 3-year-old child with uritcaria pigmentosa displaying recurrent syncope and anaphylaxis as the first presentation of systemic mastocytosis. We found data to be limited on this topic, and concluded that pediatric patients with prior diagnoses of cutaneous mastocytosis could benefit from either more aggressive screening for systemic disease or prophylactic treatment with antihistamines and rescue subcutaneous epinephrine.
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PMID:Recurrent syncope and anaphylaxis as presentation of systemic mastocytosis in a pediatric patient: case report and literature review. 1663 42

Mastocytosis is a disease characterized by an abnormal increase in mast cells. Manifestations of the disease are provoked in large part by the resultant increase in mast cell-derived mediators, which have a variety of local and systemic effects. Mastocytosis is variable in respect to the organ systems involved, clinical manifestations, and association with hematologic diseases. This has suggested the need for an improved classification scheme to allow assessment of prognosis and therapy. The heterogeneity of the disease patterns in mastocytosis strongly suggests that more than one biologic lesion may occur in the developmental sequence that leads to placement of mature mast cells in tissues.
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PMID:Classification and diagnosis of mastocytosis: current status. 1679 1

The onset of mastocytosis occurs between birth and 2 years of age in approximately 55% of all cases; an additional 10% develop the disease before the age of 15 years. Mastocytosis in these age groups differs in many respects from mastocytosis that has its onset in adulthood. The typical presentation of pediatric-onset mastocytosis consists of cutaneous manifestations: either a solitary mastocytoma, urticaria pigmentosa, or, less commonly, diffuse cutaneous mastocytosis. Particularly in infants, bullous eruptions may occur. Mastocytosis in infants and children may involve internal organs, including the bone marrow and the gastrointestinal tract, although such manifestations appear to be less common in children than in adults. Plasma histamine levels may be elevated in pediatric-onset mastocytosis. Treatment usually involves the use of H1 and H2 antihistamines to control itching and to control the hypersecretion of gastric acid that may occur. The prognosis for children with mast cell disease is variable; approximately half of the children with urticaria pigmentosa may experience resolution of lesions and symptoms by adolescence.
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PMID:Pediatric mastocytosis. 1679 3

Mastocytosis is a disease characterized by pathological mast cell accumulation and activation in tissues. Most patients with mastocytosis exhibit the D816V point mutation in the tyrosine kinase domain of the transmembrane receptor protein Kit, leading to its constitutive activation in bone marrow or lesional skin tissue. Detection of a codon 816 c-kit mutation is included as a minor diagnostic criterion in the World Health Organization's diagnostic criteria for systemic mastocytosis. Determining mutational status of the c-kit gene also has pharmacogenomic implications in patients considered for investigational mast cell cytoreductive therapies. This article reviews diagnostic and therapeutic implications of c-kit mutations as well as other less common molecular abnormalities observed in mast cell disease.
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PMID:Molecular diagnosis of mast cell disorders: a paper from the 2005 William Beaumont Hospital Symposium on Molecular Pathology. 1693 79

Mastocytosis is characterized by pathologic mast cell accumulation and activation in tissues. Establishment of objective histopathologic and molecular criteria for diagnosis of mastocytosis has allowed sensitive detection of mast cells with aberrant features in patients presenting with suspected mast cell activation symptoms. Frequent detection of the D816V c-kit tyrosine kinase mutation in mastocytosis has led to evaluation of small-molecular-weight tyrosine kinase inhibitors as mast cell cytoreductive agents. In vitro experiments, however, showed that mast cells carrying the D816V c-kit mutation were resistant to the prototypical tyrosine kinase inhibitor imatinib. Efficacy of newer generation tyrosine inhibitors in mast cell disease is currently being evaluated.
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PMID:Mastocytosis: advances in diagnosis and treatment. 1754 45

Mastocytosis is a neoplastic disease involving mast cells (MC) and their CD34+ progenitors. Symptoms in mastocytosis are caused by biological mediators released from MC and/or the infiltration of neoplastic MC in various organs, the skin and the bone marrow being predominantly involved. A WHO consensus classification for mastocytosis exists, which is widely accepted and includes three major categories: (1) Cutaneous mastocytosis (CM), a benign disease in which MC infiltration is confined to the skin, is preferentially seen in young children and exhibits a marked tendency to regress spontaneously. (2) Systemic mastocytosis (SM) which is commonly diagnosed in adults and includes four major subtypes: (i) indolent SM (ISM, the most common form involving mainly skin and bone marrow); (ii) a unique subcategory termed SM with an associated non-mast cell clonal hematological disease (SM-AHNMD); (iii) aggressive SM usually presenting without skin lesions, and (iv) MC leukemia, probably representing the rarest variant of human leukemias. (3) The extremely rare localized extracutaneous MC neoplasms, either presenting as malignancy (MC sarcoma) or as benign tumor termed extracutaneous mastocytoma. Diagnostic criteria for mastocytosis are available and are widely accepted. SM criteria include one major criterion (multifocal compact tissue infiltration by MC) and four minor criteria: (1) prominent spindling of MC; (2) atypical immunophenotype of MC with coexpression of CD2 and/or CD25 (antigens which have not been found to be expressed on normal/reactive MC); (3) activating (somatic) point mutations of the c-kit proto-oncogene usually involving exon 17, with the imatinib-resistant type D816V being most frequent, and (4) persistently elevated serum tryptase level (>20 ng/ml). To establish the diagnosis of SM, at least one major and one minor criterion, or at least three minor criteria, have to be fulfilled. The natural clinical course of mastocytosis is variable. Most patients, in particular those with CM and ISM, remain in an indolent stage over many years or even decades, while others, in particular those with aggressive SM, SM-AHNMD, or mast cell leukemia, show a progressive course, usually with a fatal outcome.
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PMID:Mastocytosis: state of the art. 1758 83

Mastocytosis is a disorder characterised by abnormal mast cell proliferation. The diverse spectrum of clinical presentations is dependent on the tissues and organs involved. We report a rare case of aggressive systemic mastocytosis presenting in utero with diffuse cutaneous involvement, and haematological abnormalities. There is little published evidence to guide treatment.
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PMID:Systemic mastocytosis with diffuse cutaneous involvement and haematological disease presenting in utero treated unsuccessfully with vincristine. 1798 56

Mastocytosis consists of a group of disorders characterized by a pathologicincrease in mast cells in tissues including skin, bone marrow, liver, spleen, andlymph nodes. Mastocytosis is a rare disease and general practitioners have limited exposure to its clinical manifestations, diagnosis, classification, and management. Moreover a complete and clear review in this field is not easy founded. Diagnosis of mastocytosis is suspected on clinical grounds and is established by histopathologic examination of involved tissues such as skin and bone marrow. The most common clinical sign of mastocytosis is the presence of typical skin lesions of urticaria pigmentosa. Most patients experience symptoms related to mast cell mediator release, and prevention of the effects of these mediators on tissues constitutes the major therapeutic goal in the management of mastocytosis. Despite recent advances in knowledge about the pathophysiology, diagnosis, and classification of mastocytosis, a curative treatment for mastocytosis does not now exist; furthermore mastocytosis is a chronic diseases with different severity grades but in all of them with an important negative impact on quality of live of patients. Management of patients within all categories of mastocytosis includes: 1. A careful counselling of patients (parents in paediatric cases) and care providers. 2. Avoidance of factors triggering acute mediator release. 3. Treatment of acute mast cell mediator release. 4. Treatment of chronic mast cell mediator release, and if indicated. 5. An attempt to treat organ infiltration by mast cells. The goal of this review is to provide a practical guide focus on diagnostic criteria for the different treatment options currently available and their management.
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PMID:[Guidelines for the diagnosis, treatment and management of mastocytosis]. 1849 77


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