Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P06126 (CD1a)
 2,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pulmonary Langerhans cell Histiocytosis is a rare granulomatous disease affecting both sexes, with greater incidence in the second and third decades of life; smoking appears to be the most important risk factor. Its etiology is unknown, although there are data indicating an uncontrolled immune response as possible cause, sustained by the Langerhans cells, antigen presenting cells for T lymphocytes, and their accumulation in the distal bronchioles; these cells express on their surface the CD1a and CD1c antigens, and the B7 molecule, essential for activating quiescent T lymphocytes. In its evolution the granuloma is characterized by the progressive reduction in the LC number, with the increase of fibrosis, surrounding and destroying the bronchiolar lumen; the remaining of the lumen, or the traction exerted by fibrous tissue on the adjacent alveolar spaces leads to the cyst development. Vascular involvement occurs frequently, and may explain the onset of pulmonary hypertension in advanced cases of the disease. The disease may be asymptomatic, or it may present with aspecific respiratory signs and symptoms, and has characteristic radiological findings, being included in the group of cysticaerial parenchymal alterations. The diagnosis could be suggested by the finding of a number of LC in BAL greater than 5%. Different therapies have been proposed, but it seems that the most important measure is smoking cessation.
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PMID:Pulmonary Langerhans cell Histiocytosis. 1158 96

Langerhans cell histiocytosis with only pulmonary involvement is a rare, probably reactive, proliferating disease of young and smoking persons. During last 7 years 7 cases of Langerhans cell histiocytosis were diagnosed in our Department. There were 6 men and one woman. Mean age was about 32 years. All of them were smokers. Intensity and duration of smoking varied widely from 5 to 40 cigarettes/day and 5 to 30 years respectively. The most frequent symptoms were dry cough, dyspnea. Two patients had no complaints. Pneumothorax preceded diagnosis in 4 patients. Duration of symptoms varied from 3 months to 10 years--in a patient who was previously diagnosed as allergic alveolitis. All patients had typical radiological and HRCT appearance of disseminated nodular lesions with small cysts formation with sparing of costo-phrenic angles. Three of patients had very advanced disease with cystic lung destruction. Restriction was noticed in those three patients. Diminished values of compliance and diffusing capacity for CO was observed in four patients. Bronchoscopy examination of bronchial tree revealed only slight inflammation in all patients. BAL was assessed for presence of CD1a cells in 6 cases, but in no one patient elevated number of those cells was noticed (2-4.5%). In all patients open lung biopsy was performed, and typical histologic appearance was observed. In six patients also CD1a positive cells were revealed in lung specimens.
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PMID:[Pulmonary Langerhans cell histiocytosis--clinicopathologic analysis of 7 cases]. 1173 89

Antigen-presenting cells (APCs) are strategically placed in all anatomic sites with high antigen exposure such as the respiratory system. The aim of this study was to evaluate phenotypic and functional properties of APCs from the lung (L-Cs), mediastinal lymph node (LN-Cs) and bronchoalveolar lavage cells (BAL-Cs). The APCs were first analyzed based on forward scatter and side scatter profiles and the selection of MHC-II(high)CD172a(+) cells (referred to as APCs); then the expression of CD1a, CD163, CD206, CD16 and CD11R3 was evaluated in the APCs. The results showed that CD1a, CD163 and CD206 were differentially expressed among L-Cs, LN-Cs and BAL-Cs, suggesting the phenotype MHC-II(high)CD172a(+)CD1a(low/-)CD163(low)CD206(-) for L-Cs and MHC-II(high)CD172a(+)CD1a(+)CD163(low/-)CD206(+) for LN-Cs. BAL-Cs were MHC-II(high)CD172a(+)CD1a(-)CD163(high)CD206(+/-). The functional characteristics of L-Cs and LN-Cs were different from those of BAL-Cs, confirming that L-Cs and LN-Cs resemble specialized APCs. In conclusion, we present the characterization of APCs from L-Cs, LN-Cs and BAL-Cs of the porcine respiratory system.
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PMID:Characterization of antigen-presenting cells from the porcine respiratory system. 2595 37