scholarly journals Light and electron microscopy characteristics of the muscle of patients with SURF1 gene mutations associated with Leigh disease

2007 ◽  
Vol 61 (4) ◽  
pp. 460-466 ◽  
Author(s):  
M Pronicki ◽  
E Matyja ◽  
D Piekutowska-Abramczuk ◽  
T Szymańska-Dębińska ◽  
A Karkucińska-Więckowska ◽  
...  

Aims:Leigh syndrome (LS) is characterised by almost identical brain changes despite considerable causal heterogeneity. SURF1 gene mutations are among the most frequent causes of LS. Although deficiency of cytochrome c oxidase (COX) is a typical feature of the muscle in SURF1-deficient LS, other abnormalities have been rarely described. The aim of the present work is to assess the skeletal muscle morphology coexisting with SURF1 mutations from our own research and in the literature.Methods:Muscle samples from 21 patients who fulfilled the criteria of LS and SURF1 mutations (14 homozygotes and 7 heterozygotes of c.841delCT) were examined by light and electron microscopy.Results:Diffuse decreased activity or total deficit of COX was revealed histochemically in all examined muscles. No ragged red fibres (RRFs) were seen. Lipid accumulation and fibre size variability were found in 14 and 9 specimens, respectively. Ultrastructural assessment showed several mitochondrial abnormalities, lipid deposits, myofibrillar disorganisation and other minor changes. In five cases no ultrastructural changes were found. Apart from slight correlation between lipid accumulation shown by histochemical and ultrastructural techniques, no other correlations were revealed between parameters investigated, especially between severity of morphological changes and the patient’s age at the biopsy.Conclusion:Histological and histochemical features of muscle of genetically homogenous SURF1-deficient LS were reproducible in detection of COX deficit. Minor muscle changes were not commonly present. Also, ultrastructural abnormalities were not a consistent feature. It should be emphasised that SURF1-deficient muscle assessed in the light and electron microscopy panel may be interpreted as normal if COX staining is not employed.

2016 ◽  
Vol 36 (suppl 1) ◽  
pp. 89-94 ◽  
Author(s):  
Luciana S. Simões ◽  
Rose E.G. Rici ◽  
Phelipe O. Favaron ◽  
Taís Harumi de Castro Sasahara ◽  
Rodrigo S.N. Barreto ◽  
...  

Abstract: al for both, the establishment of appropriate management systems, and for the use of new species as animal models. In this study, we used light and electron microscopy to characterize the sexual development stages of the guinea pig (Cavia porcellus) in specimens of 30, 45 and 90 days of age. We observed the differentiation of spermatocytes only through transmission electron microscopy in the leptotene, zygotene and pachytene phases of meiosis, in 30-day-old animals. During puberty, there was differentiation of the germinative epithelium and formation of the acrosome. Spermatozoa, however, were not detected. Thus, we could infer that puberty happens after 45 days of age. Sexual maturity was evident in 90-day-old specimens. Our results showed that changes in the testicular germinative epithelium during the postnatal sexual development in guinea pig led to morphological changes, including the ones related to the development of Leydig and Sertoli cells, which are directly related to puberty. In this work, we provide new morphological subsidies for a better understanding of reproductive parameters of this species, enabling its use as an animal model in the field of the reproductive biology.


1977 ◽  
Vol 25 (2) ◽  
pp. 207 ◽  
Author(s):  
DE Hollis ◽  
AG Lyne

Eleven embryos, ranging from partly to fully bilaminar blastocysts, were obtained from the bandicoots I. macrourus and P. nasuta and were examined by light and electron microscopy. The morphological changes which occurred during the differentiation of the endoderm and ectoderm are described. The shell membrane was thinner than it was in unilaminar blastocysts and had a deposit of material of irregular thickness on its outer surface. The mucoid coat and zona pellucida were absent or discontinuous. Endoderm formation was first observed in blastocysts about 1.0 mm in diameter. Cells migrated inwards from regions of thickened protoderm to form a continuous layer of similar flattened cells - the endoderm - beneath the protoderm, which then became the ectoderm. The blastocysts were fully bilaminar when they were 1.5-1.9 mm in diameter. At this stage the ectoderm was composed of two distinct regions, an embryonic region of cuboidal cells and a non-embryonic region of flattened cells resembling the cells of the endoderm. The formation of the endoderm in bandicoots closely resembles that described in other marsupials, except the American opossum Didelphis virginiana in which endoderm cells are released into the blastocoele before they form a continuous layer.


Author(s):  
Horiya H. Al-Azri ◽  
Taher Ba-Omar ◽  
Abdulkadir Elshafie ◽  
Michael J Barry

Aflatoxin B1 (AFB1) is a mycotoxin which can cause serious toxicity to animals and humans.  The aim of this study was to investigate the effects of AFB1 in Aphanius dispar fish and measure residues in tissues after in vivo exposure. Aphanius dispar were fed diets containing 50, 100, 150 and 200 µg AFB1/kg for 10, 20 and 30 days. At the end of the experiment, the liver and gills were dissected out and processed for light and electron microscopy. During the experiment, no external changes or unusual behavior were observed in the fish. Histopathological and ultrastructural changes in liver appeared under all four treatments: 50, 100, 150 and 200 µg AFB1/kg. Gill tissues were affected at high doses of 100,150 and 200 µg AFB1/kg. Accumulation of AFB1 residues in liver and gill tissues was found to be related to a dose and duration of exposure.  


Author(s):  
D.L. Friesen ◽  
A. Singh ◽  
M.E. Hitt

Thiacetarsamide is an arsenic-containing drug used in the treatment of heartworm in dogs. The effective antihelmintic dose is toxic to the host animal. Acetylcysteine decreases the hepatotoxicity of some compounds by forming a conjugate with toxic metabolites of the compound. The purpose of this study was to evaluate the effectiveness of a cytoprotectant for hepatocytes in dogs treated with therapeutic levels of thiacetarsamide.Eighteen dogs were divided randomly into two groups. All dogs were given four doses of thiacetarsamide over two days. Nine dogs were given 10% acetylcysteine 15 min prior to each dose of thiacetarsamide. Needle biopsies of the liver were taken from each dog prior to the treatment and again one week post-treatment. The biopsies were fixed in 2% gluraraldehyde in phosphate buffer, pH 7.3, post-fixed in 1% osmium tetroxide, and processed for electron microscopy. Semithin and thin sections of the liver were examined by light and electron microscopy, respectively, for histopathologic and ultrastructural changes. The specimens were coded and the sample treatment was not known to the researchers at the time of observation.


Parasitology ◽  
2014 ◽  
Vol 141 (12) ◽  
pp. 1638-1645 ◽  
Author(s):  
L. F. NIMRI ◽  
H. N. LANNERS

SUMMARYNone of the few animal models proposed for the study of human quartan malaria nephritic syndrome have shown complete pathological findings that are similar to those seen in humans. This study investigated the histopathological changes in kidneys in 10 Plasmodium inui infected Macaca mulatta monkeys by light and electron microscopy in order to develop a suitable animal model for human quartan malaria. Ten healthy adult rhesus monkeys were infected with P. inui and clinical chemistry and haematologic tests were done before and after infection. A renal biopsy sample was collected before infection as a baseline control and another biopsy was collected after infection. Histopathological changes examined by light and transmission electron microscopy (TEM) revealed abnormalities in all infected monkeys to variable degrees. Several electron-dense discrete or diffused mesangial deposits, and increase in mesangial cells and matrix were associated with the morphological changes observed by light microscope. This pattern is consistent with membranoproliferative glomerulonephritis type reported in humans infected with Plasmodium malariae. Results strongly support that the P. inui-infected rhesus monkey develop an immune-complex-mediated glomerulonephritis in the course of the infection. Therefore, this experimental model represents a useful tool to better understand the different parameters and the consequences of quartan malaria infections comparable to situations in humans.


2014 ◽  
Vol 26 (7) ◽  
pp. 991 ◽  
Author(s):  
Verónica Filippa ◽  
Daiana Godoy ◽  
Edith Perez ◽  
Fabian Mohamed

The aims of the present study were to determine whether castration results in quantitative immunohistochemical changes in androgen receptors (AR), LH-immunoreactive (IR) cells and FSH-IR cells, and to analyse the colocalisation of AR and gonadotropins in the pituitary pars distalis (PD) of viscachas. Pituitaries were processed for light and electron microscopy. AR-IR, LH-IR and FSH-IR cells were detected by immunohistochemistry. In morphometric studies, the percentage of AR-IR, LH-IR, FSH-IR, LH-IR/AR-IR and FSH-IR/AR-IR cells was determined. In intact viscachas, AR were distributed throughout the PD; they were numerous at the caudal end, with intense immunostaining. LH-IR cells and FSH-IR cells were found mainly in the ventral region and at the rostral end of the PD. Approximately 45%–66% of LH-IR cells and 49%–57% of FSH-IR cells expressed AR in the different zones of the PD. In castrated viscachas, there was a significant decrease in the percentage of AR-IR, LH-IR, FSH-IR, and FSH-IR/AR-IR cells. Some pituitary cells from castrated viscachas also exhibited ultrastructural changes. These results provide morphological evidence that gonadal androgens are directly related to the immunolabelling of AR, LH and FSH. Moreover, the colocalisation of AR and FSH is most affected by castration, suggesting the existence of a subpopulation of gonadotrophs with different regulatory mechanisms for hormonal synthesis, storage and secretion.


2021 ◽  
Vol 5 (1) ◽  
pp. 5-16
Author(s):  
V. M. Tsyrkunov ◽  
◽  
N. I. Prokopchik ◽  
V. P. Andreev ◽  
◽  
...  

The review presents literature data on the main pathomorphological features of autoimmune hepatitis (AIH) and the findings of light and electron microscopy in the form of photographs and descriptions of different variants of hepatic morphological changes in patients with AIH. Updates to the sections of the AIH classification concerning the etiology and origin of AIH are suggested, clinical examples are given with a description of the morphology features according to the classification specified by the authors.


2020 ◽  
Vol 79 (5) ◽  
pp. 508-517
Author(s):  
Min-Cheol Lee ◽  
Ra Gyung Kim ◽  
Taebum Lee ◽  
Jo-Heon Kim ◽  
Kyung-Hwa Lee ◽  
...  

Abstract Diaschisis has been described as functional depression distant to the lesion. A variety of neuroscientific approaches have been used to investigate the mechanisms underlying diaschisis. However, few studies have examined the pathological changes in diaschisis at ultrastructural level. Here, we used a rat model of capsular infarct that consistently produces diaschisis in ipsilesional and contralesional motor and sensory cortices. To verify the occurrence of diaschisis and monitor time-dependent changes in diaschisis, we performed longitudinal 2-deoxy-2-[18F]-fluoro-d-glucose microPET (FDG-microPET) study. We also used light and electron microscopy to identify the microscopic and ultrastructural changes at the diaschisis site at 7, 14, and 21 days after capsular infarct modeling (CIM). FDG-microPET showed the occurrence of diaschisis after CIM. Light microscopic examinations revealed no significant histopathological changes at the diaschisis site except a mild degree of reactive astrogliosis. However, electron microscopy revealed swollen, hydropic degeneration of axial dendrites and axodendritic synapses, although the neuronal soma (including nuclear chromatin and cytoplasmic organelles) and myelinated axons were relatively well preserved up to 21 days after injury. Furthermore, number of axodendritic synapses was significantly decreased after CIM. These data indicate that a circumscribed subcortical white-matter lesion produces ultrastructural pathological changes related to the pathogenesis of diaschisis.


2022 ◽  
Vol 23 (2) ◽  
pp. 854
Author(s):  
Ruiyi Ren ◽  
Anne A. Humphrey ◽  
David L. Swain ◽  
Haiyan Gong

We investigated whether an inverse relationship exists between intraocular pressure (IOP) and effective filtration area (EFA) in the trabecular meshwork (TM) in a steroid-induced ocular hypertensive (SIOH) mouse model and the morphological changes associated with the reduction of EFA. C57BL/6 mice (n = 15 per group) received either 0.1% dexamethasone (DEX) or saline eye drops twice daily for five weeks. IOP was measured weekly. Fluorescent tracers were injected into the anterior chamber to label EFA at the endpoint. Injected eyes were fixed and processed for confocal microscopy. EFA in the TM was analyzed. Light and electron microscopy were performed in high- and low-tracer regions of six eyes per group. The mean IOP was ~4 mm Hg higher in DEX-treated than saline-treated control eyes (p < 0.001) at the endpoint. EFA was reduced in DEX-treated eyes compared to controls (p < 0.01) and negatively correlated with IOP (R2 = 0.38, p = 0.002). Reduced thickness of juxtacanalicular tissue (JCT) and increased abnormal extracellular matrix in the JCT were found to be associated with reduced EFA. Our data confirm the inverse relationship between EFA and IOP, suggesting that morphological changes in the JCT contribute to the reduction of EFA, thus elevating IOP in SIOH mouse eyes.


1994 ◽  
Vol 9 (2) ◽  
pp. 48-54 ◽  
Author(s):  
M. Tronnier ◽  
W. Schmeller ◽  
H. H. Wolff

Objectives: To demonstrate morphological changes in lipodermatosclerosis (LDS) and venous ulcers by histology, immunohistochemistry, electron microscopy and immunoelectron microscopy. Design: Single patient group study in patients with trophic skin changes in chronic venous insufficiency. Setting: Department of Dermatology, Medical University of Lübeck. Patients: Ten patients with venous leg ulcers. Interventions: Biopsies were taken from areas of LDS and compared with clinically normal-appearing skin of the affected leg and with ulcer tissue. Main outcome measures: Comparison of the morphological features on light and electron microscopy. Results: Superficial dermis. Histologically, the ulcer tissue and LDS skin show dilated tortuous vessels in a glomerulus-like arrangement in the superficial parts of the dermis. Ultrastructurally, the superficial vessels are surrounded by a cuff, which contains amorphous and basal membrane material and is most pronounced in LDS. Immunofluorescence studies reveal ill-defined perivascular staining after incubation with antibodies against fibrin(ogen), laminin and type IV collagen. The exact ultrastructural localization of type IV collagen within the perivascular cuff is observed by immunoelectron microscopy. Deep dermis. In deeper parts of the dermis, the vessels of both ulcer tissue and LDS are surrounded by cellular cuffs with pericytes, fibroblasts and compact collagen bundles. Conclusions: We suggest that the severe morphological changes in LDS and ulcer tissue play an important role in the pathogenesis of venous ulceration.


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