Bcl-2 Expression in Langerhans' Cell Histiocytosis

1998 ◽  
Vol 1 (3) ◽  
pp. 210-215 ◽  
Author(s):  
Van H. Savell ◽  
Todd Sherman ◽  
Richard H. Scheuermann ◽  
Abdul M. Siddiqui ◽  
Linda R. Margraf
Keyword(s):  
In Situ Hybridization ◽  
Langerhans Cells ◽  
Langerhans Cell Histiocytosis ◽  
Germ Line ◽  
Normal Skin ◽  
Protein Product ◽  
Langerhans Cell ◽  
Genomic Rearrangement ◽  
Southern Analysis

Langerhans' cell histiocytosis (LCH) is an abnormal accumulation of dendritic histiocytes of unknown pathogenesis. It has recently been shown to be a clonal process. Bcl-2 is a proto-oncogene whose protein product is known to inhibit apoptosis. The overexpression of bcl-2 has been demonstrated in a number of neoplasms, presumably prolonging the survival of the neoplastic cells. We examined the expression of bcl-2 in normal Langerhans' cells in the skin and in LCH by immunohistochemistry for protein and in situ hybridization for mRNA to see if it could be implicated in the pathogenesis of this disorder. Additionally, we performed Southern analysis to determine if genomic rearrangement of the bcl-2 gene occurs in cases of LCH. Bcl-2 was not detected in normal skin Langerhans' cells. Eleven of thirteen cases of LCH demonstrated bcl-2 protein expression in the cytoplasm of the Langerhans' cells by immunohistochemistry, while 12 of 13 cases had evidence of bcl-2 mRNA by in situ hybridization. Southern analysis revealed a germ-line configuration of the bcl-2 gene in the five cases studied. These findings suggest that bcl-2 expression is present and up-regulated in pathologic Langerhans' cells, however, this overexpression does not appear to be due to genomic rearrangement.

Comet and cup genes in Drosophila spermatogenesis: the first demonstration of post-meiotic transcription

2008 ◽  
Vol 36 (3) ◽  
pp. 540-542 ◽  
Author(s):  
Carine Barreau ◽  
Elizabeth Benson ◽  
Helen White-Cooper
Keyword(s):  
In Situ Hybridization ◽  
Expression Pattern ◽  
Reverse Transcription ◽  
Protein Product ◽  
Rt Pcr ◽  
Pcr Assay ◽  
Single Cyst ◽  
Reverse Transcription Pcr

Post-meiotic transcription is widespread in mammalian spermatogenesis, but is generally believed to be absent from Drosophila spermatogenesis. Genes required during meiosis, in early spermatids or later in spermiogenesis are typically transcribed in primary spermatocytes in Drosophila. Their mRNAs are then stored in the cytoplasm until the protein product is needed. Recently, using in situ hybridization, we identified 17 Drosophila genes, collectively named ‘comets’ and ‘cups’, whose mRNAs are most abundant in, and localize to the distal ends of, elongating spermatids. Using a single-cyst quantitative RT–PCR (reverse transcription–PCR) assay, we confirmed this unusual expression pattern and conclusively demonstrate the existence of post-meiotic transcription in Drosophila spermatids. We found that transcription of comets and cups occurs just before protamines can be detected in spermatid nuclei.

scholarly journals Germ line-specific DNA sequences are present on all five micronuclear chromosomes in Tetrahymena thermophila.

1983 ◽  
Vol 3 (11) ◽  
pp. 1909-1919 ◽  
Author(s):  
K M Karrer
Keyword(s):  
In Situ Hybridization ◽  
Dna Sequences ◽  
Tetrahymena Thermophila ◽  
Germ Line ◽  
Plasmid Vector ◽  
Ciliated Protozoan ◽  
Specific Sequence ◽  
Individual Probe ◽  
Vector Pbr322

The development of the macronucleus from the zygotic micronucleus in the ciliated protozoan Tetrahymena spp. involves the elimination of specific DNA sequences (M. C. Yao and M. Gorovsky, Chromosoma 48:1-18 1974). The present study demonstrates that micronucleus-specific DNA is present on all five of the micronuclear chromosomes. Fragments of micronuclear DNA from Tetrahymena thermophila were cloned in the plasmid vector pBR322. A procedure was developed to examine the organization of the cloned sequences in micro- and macronuclear DNA without nick translating each individual probe. Twenty-three percent of randomly selected DNA sequences examined by this method were micronucleus (germ line) specific. They were all members of families of repeated sequences. Hybridization of six micronucleus-specific DNA sequences to micronuclear DNA from nullisomic strains of T. thermophila, which are lacking one or more pairs of chromosomes in the micronucleus, suggested that these sequences are present on several chromosomes. One micronucleus-specific sequence was shown by in situ hybridization to be present on all five of the micronuclear chromosomes.

Clonal proliferation of Langerhans cells in Langerhans cell histiocytosis

The Lancet ◽  
1994 ◽  
Vol 343 (8900) ◽  
pp. 767-768 ◽  
Author(s):  
R.C. Yu ◽  
A.C. Chu ◽  
C. Chu ◽  
L. Buluwela
Keyword(s):  
Langerhans Cells ◽  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Clonal Proliferation

Gene expression analysis of dendritic/Langerhans cells and Langerhans cell histiocytosis

2006 ◽  
Vol 209 (4) ◽  
pp. 474-483 ◽  
Author(s):  
R Rust ◽  
J Kluiver ◽  
L Visser ◽  
G Harms ◽  
T Blokzijl ◽  
...  
Keyword(s):  
Gene Expression ◽  
Expression Analysis ◽  
Langerhans Cells ◽  
Langerhans Cell Histiocytosis ◽  
Gene Expression Analysis ◽  
Langerhans Cell

scholarly journals Adeno-Associated Virus as a Vector for Liver-Directed Gene Therapy

1998 ◽  
Vol 72 (12) ◽  
pp. 10222-10226 ◽  
Author(s):  
Weidong Xiao ◽  
Scott C. Berta ◽  
Min Min Lu ◽  
A. David Moscioni ◽  
John Tazelaar ◽  
...  
Keyword(s):  
Gene Therapy ◽  
In Situ Hybridization ◽  
Immunohistochemical Analysis ◽  
Southern Analysis ◽  
Portal Circulation ◽  
Adeno Associated Virus ◽  
Diploid Genome ◽  
Immunodeficient Mice

ABSTRACT Factors relevant to the successful application of adeno-associated virus (AAV) vectors for liver-directed gene therapy were evaluated. Vectors with different promoters driving expression of human α-1-antitrypsin (α-1AT) were injected into the portal circulation of immunodeficient mice. α-1AT expression was stable but dependent on the promoter. Southern analysis of liver DNA revealed approximately 0.1 to 2.0 provirus copies/diploid genome in presumed head-to-tail concatamers. In situ hybridization and immunohistochemical analysis revealed expression in approximately 5% of hepatocytes clustered in the pericentral region. These results support the use of AAV as a vector for diseases treatable by targeting of hepatocytes.

scholarly journals Germ line-specific DNA sequences are present on all five micronuclear chromosomes in Tetrahymena thermophila

1983 ◽  
Vol 3 (11) ◽  
pp. 1909-1919
Author(s):  
K M Karrer
Keyword(s):  
In Situ Hybridization ◽  
Dna Sequences ◽  
Tetrahymena Thermophila ◽  
Germ Line ◽  
Plasmid Vector ◽  
Ciliated Protozoan ◽  
Specific Sequence ◽  
Individual Probe ◽  
Vector Pbr322

The development of the macronucleus from the zygotic micronucleus in the ciliated protozoan Tetrahymena spp. involves the elimination of specific DNA sequences (M. C. Yao and M. Gorovsky, Chromosoma 48:1-18 1974). The present study demonstrates that micronucleus-specific DNA is present on all five of the micronuclear chromosomes. Fragments of micronuclear DNA from Tetrahymena thermophila were cloned in the plasmid vector pBR322. A procedure was developed to examine the organization of the cloned sequences in micro- and macronuclear DNA without nick translating each individual probe. Twenty-three percent of randomly selected DNA sequences examined by this method were micronucleus (germ line) specific. They were all members of families of repeated sequences. Hybridization of six micronucleus-specific DNA sequences to micronuclear DNA from nullisomic strains of T. thermophila, which are lacking one or more pairs of chromosomes in the micronucleus, suggested that these sequences are present on several chromosomes. One micronucleus-specific sequence was shown by in situ hybridization to be present on all five of the micronuclear chromosomes.

Langerhans Cell Histiocytosis: Back to Histiocytosis X

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. SCI-8-SCI-8
Author(s):  
Carl E. Allen
Keyword(s):  
Dendritic Cells ◽  
Dendritic Cell ◽  
Langerhans Cells ◽  
Langerhans Cell Histiocytosis ◽  
Conflicts Of Interest ◽  
Langerhans Cell ◽  
Histiocytosis X ◽  
Specific Gene ◽  
Myeloid Dendritic Cell ◽  
Epidermal Langerhans Cells

Abstract Abstract SCI-8 Langerhans cell histiocytosis (LCH) is a disorder characterized by inflammatory lesions that include pathologic CD207+ dendritic cells. LCH has pleotropic clinical presentations ranging from single lesions cured by curettage to potentially fatal multisystem disease. The first descriptions of LCH, including Hand-Schüller-Christian disease and Letterer-Siwe disease, were based on anatomic location and extent of the lesions. Despite clinical heterogeneity, LCH lesions are generally indistinguishable by histology, which led to the notion that the spectrum of clinical manifestations represents a single disorder, histiocytosis X. The designation “Langerhans cell histiocytosis” was subsequently proposed with discovery of cytoplasmic Birbeck granules in the pathologic infiltrating dendritic cells in histiocytosis X lesions, a feature shared by epidermal Langerhans cells. The etiology of LCH remains elusive, and debate of LCH as an inflammatory versus malignant disorder remains unresolved. However, recent discoveries question the model of LCH arising from transformed or pathologically activated epidermal Langerhans cells. We found cell-specific gene expression signature in CD207+ dendritic cells within LCH lesions to be more consistent with immature myeloid dendritic cell precursors than epidermal Langerhans cells. Furthermore, recent mouse studies demonstrate that CD207+ is more promiscuous than previously appreciated. Langerin (CD207) expression can be induced in many dendritic cell lineages, supporting the plausibility of a spectrum of candidates for an LCH cell of origin, including circulating dendritic cell precursors. Finally, recurrent activating BRAF mutations in LCH lesions suggest a role for a hyperactive RAS pathway in LCH pathogenesis, and possibly in normal dendritic cell development. This presentation will discuss the historical background and recent advances in LCH biology, along with a proposal to reframe “histiocytosis X” as a myeloid neoplasia caused by aberrant maturation and migration of myeloid dendritic cell precursors. Disclosures: No relevant conflicts of interest to declare.

Pulmonary Langerhans’ cell histiocytosis

2020 ◽  
pp. 4256-4257
Author(s):  
S. J. Bourke
Keyword(s):  
Bone Marrow ◽  
Dendritic Cells ◽  
Genital Tract ◽  
Langerhans Cells ◽  
Langerhans Cell Histiocytosis ◽  
Female Genital Tract ◽  
Cyst Formation ◽  
Langerhans Cell ◽  
Pulmonary Langerhans Cell Histiocytosis ◽  
Systemic Symptoms

Pulmonary Langerhans’ cell histiocytosis is characterized by a reactive monoclonal proliferation of activated histiocytes in the distal bronchioles, resulting in inflammatory nodules, cyst formation, and fibrosis. Langerhans’ cells are a particular type of histiocyte derived from dendritic cells in the bone marrow. They normally migrate in the blood to the squamous epithelium of the skin, lungs, gastrointestinal, and female genital tract, where they are involved in antigen presentation to T cells. It presents with cough, breathlessness, and (sometimes) systemic symptoms. Chest radiography and CT typically show nodules which then cavitate and may rupture, causing pneumothorax. Corticosteroids and/or cytotoxic drugs are of some benefit, and lung transplantation is an option for progressive disease.

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