The TSC-2 product tuberin is expressed in lymphangioleiomyomatosis and angiomyolipoma

The TSC‐2 product tuberin is expressed in lymphangioleiomyomatosis and angiomyolipoma Aims: Lymphangioleiomyomatosis is categorized by proliferation of abnormal smooth muscle cells (LAM cells) in the lungs and lymphatics and the presence of angiomyolipomas. Recently mutations in the tuberous scleros...

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Veröffentlicht in:Histopathology Jg. 40; H. 5; S. 458 - 463
Hauptverfasser: Johnson, S R, Clelland, C A, Ronan, J, Tattersfield, A E, Knox, A J
Format: Journal Article
Sprache:Englisch
Veröffentlicht: Oxford UK Blackwell Science Ltd 01.05.2002
Blackwell
Schlagworte:
Adult
Aged
angiomyolipoma
Angiomyolipoma - metabolism
Angiomyolipoma - pathology
Biological and medical sciences
Female
Humans
Immunohistochemistry
Kidney Neoplasms - metabolism
Kidney Neoplasms - pathology
Kidneys
Lung Neoplasms - metabolism
Lung Neoplasms - pathology
lymphangioleiomyomatosis
Lymphangioleiomyomatosis - metabolism
Lymphangioleiomyomatosis - pathology
Male
Medical sciences
Middle Aged
Nephrology. Urinary tract diseases
Pneumology
Repressor Proteins - biosynthesis
tuberin
tuberous sclerosis
Tuberous Sclerosis Complex 2 Protein
Tumor Suppressor Proteins
Tumors of the respiratory system and mediastinum
Tumors of the urinary system
tumour suppressor
Kidney disease
Diffuse
Human
Immunohistochemistry
Lung disease
Lymphangiomatosis
Urinary system disease
Respiratory disease
Leiomyomata
Gene expression
Carcinogenesis
Bronchopulmonary
Kidney
Pathology
Gene
Tuberin
Bronchus disease
Benign neoplasm
Angiomyolipoma
Mutation
Bourneville syndrome
Tumor suppressor gene
ISSN:0309-0167, 1365-2559
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Zusammenfassung:The TSC‐2 product tuberin is expressed in lymphangioleiomyomatosis and angiomyolipoma Aims: Lymphangioleiomyomatosis is categorized by proliferation of abnormal smooth muscle cells (LAM cells) in the lungs and lymphatics and the presence of angiomyolipomas. Recently mutations in the tuberous sclerosis complex‐2 gene (TSC‐2) have been described in LAM cells and angiomyolipomas. The TSC‐2 protein tuberin is a tumour suppressor and its loss may result in cellular proliferation. We used immunohistochemistry to test the hypothesis that uncontrolled cellular proliferation in lymphangioleiomyomatosis is the result of reduced tuberin protein expression. Methods and results: Tissue from normal lung, normal kidney, lymphangioleiomyomatosis and angiomyolipomas was immunostained with three separate anti‐tuberin antibodies. Tuberin staining in normal tissues was similar to that previously described. Surprisingly, tuberin was strongly expressed in the LAM cells of all cases of lymphangioleiomyomatosis and angiomyolipoma at a greater level than in normal smooth muscle cells. The perivascular cells of angiomyolipomas, however, did not stain for tuberin. Conclusions: Our results suggest that a loss of tuberin protein in LAM cells is not the cause of the cellular proliferation seen in lymphangioleiomyomatosis. Lymphangioleiomyomatosis may result either from the expression of a mutant tuberin with abnormal function, as a result of mutations in functionally related proteins, or from more than one mechanism.
Bibliographie:ark:/67375/WNG-51MBKBZR-4
istex:DF4CE1CC4EA3581CAAEDD1B13F666CFB77764B37
ArticleID:HIS1394
ObjectType-Article-1
SourceType-Scholarly Journals-1
ObjectType-Feature-2
content type line 23
ISSN:0309-0167
1365-2559
DOI:10.1046/j.1365-2559.2002.01394.x