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G Pezeshkpour

Publications and source records attributed to G Pezeshkpour.

15 recordsLinked to original sources
PubMed1992

Molecular analysis of the myoadenylate deaminase deficiencies.

Myoadenylate deaminase (mAMPD) deficiency in a clinically heterogeous metabolic myopathy consisting of primary (inherited) and secondary (acquired) forms based on a variety of clinical and laboratory findings. To provide a basis for delineating the underlying molecular defects in mAMPD deficiency, and as a means to test the proposal for multiple forms of the resulting disease, Northern blot analyses were performed with RNA isolated from individual patients with classified primary and secondary deficiency utilizing human mAMPD cDNA probes isolated from adult skeletal muscle libraries. Analysis of nine patients with primary mAMPD deficiency indicates normal abundance of mAMPD transcript. No immunoreactive mAMPD polypeptide is detected in Western blot analyses of skeletal muscle extracts prepared from these patients. Specificity to mAMPD is demonstrated by normal creatine kinase (CK) activities and M-creatine kinase (M-CK) transcript abundance. Similar analyses of four individuals with secondary mAMPD deficiency reveal heterogeneity in this subgroup of patients. Whereas two of these patients exhibit normal mAMPD transcript abundance, two others associated with inflammatory myopathy display reductions in mAMPD and M-CK transcript abundance. Examination of tissue sections derived from the same biopsies utilized in the isolation of RNA demonstrates the integrity of the skeletal muscle in those patients with associated inflammatory myopathy. Combined, these data support the proposal for multiple forms of mAMPD deficiency, and indicate that the primary condition is most commonly characterized by specific point mutations or small deletions/rearrangements in the ampd1 gene, whereas some patients with secondary mAMPD deficiency display more generalized aberrations in gene expression.

AMP Deaminase↗
PubMed1991

Ultrastructural characteristics and DNA immunocytochemistry in human immunodeficiency virus and zidovudine-associated myopathies.

Electron microscopic features of muscle biopsies from 13 human immunodeficiency (HIV)-positive patients who had myopathy while receiving zidovudine (AZT) were compared with biopsies from five patients with HIV-induced myopathy who were not treated with AZT. All specimens showed disorganization of the myofibrillar structures, along with a varying degree of nemaline (rod) bodies, vacuolization, inflammation, and endothelial tubuloreticular profiles. One untreated and all AZT-treated patients had cytoplasmic bodies, which in the latter were abundant, large, and irregular. Two untreated patients had a peculiar osmiophilic destruction of the muscle fibers, with numerous tubuloreticular profiles in the endothelial cells and brisk inflammation that included lymphoplasmatoid cells. The AZT-treated group had ubiquitous abnormal mitochondria that complemented the presence of ragged red fibers seen by light microscopy. There was subsarcolemmal proliferation of mitochondria, with marked variation in size and shape and proliferation or disorganization of their cristae. Paracrystalline inclusions were seen in one patient. Blind re-examination of the electron micrographs showed abnormal mitochondria that readily distinguished patients with AZT-associated myopathy from those with untreated HIV-induced myopathy. Immunocytochemistry using antibodies to single- and double-stranded DNA revealed severe reduction of mitochondrial DNA compared with the normal nuclear DNA. Although the myopathies associated with HIV and AZT share common myopathologic features, the mitochondrial abnormalities are unique to the AZT-treated patients. Since mitochondrial DNA is specifically reduced, the structural changes noted on electron microscopy are probably associated with mitochondrial dysfunction. Zidovudine, a DNA chain terminator that inhibits the mitochondrial gamma-DNA polymerase, is toxic to muscle mitochondria.

Adult↗
PubMed1991

The nucleus basalis of Meynert, senile plaques, and intellectual impairment in schizophrenia.

The large, hyperchromic, cholinergic neurons of the nucleus basalis of Meynert (nbM) and the presence of senile plaques were quantified in postmortem brain tissue from 10 intellectually impaired schizophrenic patients, seven intellectually intact schizophrenic patients, seven control subjects, and three patients with Alzheimer's disease. The two groups of schizophrenic patients did not show any significant differences when compared with the control group in nbM cell density or in plaque frequency. The Alzheimer's disease patients showed the expected decrease in nbM neuronal density and increase in plaques compared with the controls. The data suggest that compromised cognitive function in schizophrenia is not associated with diffuse neuropathology of the basal forebrain cholinergic system.

Acetylcholine↗
PubMed1987

Peripheral neuropathy in mitochondrial disease.

Clinical, electrophysiological, histological and biochemical studies of two patients with mitochondrial disease revealed a moderately advanced axonal neuropathy with mitochondrial paracrystalline inclusions in Schwann cells, fibroblasts and muscle fibers. In addition there was a myopathy, and the activity of muscle cytochrome c oxidase was diminished by more than 50%. There were electrophysiological signs of myopathy, neuropathy and failure of excitation-contraction coupling in both patients. The partial enzyme deficiency raises some questions as to its pathogenetic role in these neuromyopathies.

Adult↗
PubMed1987

Calcium uptake in frozen muscle biopsy sections compared with other predictors of malignant hyperthermia susceptibility.

The frozen-section Ca45 uptake procedure of Mabuchi and Sreter has been applied to 51 frozen human muscle biopsies previously tested for halothane and for caffeine contracture responses. In preliminary tests with cat muscle, uptake varied with skeletal muscle fiber type, was absent from smooth muscle, and was blocked by agents which damage or extract sarcoplasmic reticulum. In the human biopsies, however, the calcium uptake values showed no significant correlation with any of the following parameters: time of frozen-biopsy storage up to 5 yr; fiber type distribution; positive versus negative response in the halothane contracture test; positive versus negative response in the caffeine contracture test; positive versus negative response in the combined halothane/caffeine contracture test; and presence of a documented clinical episode of malignant hyperthermia. In contrast, a positive halothane or caffeine contracture test each correlated highly with the presence of a prior clinical episode. We conclude that the evidence fails to support the use of the Ca45 uptake procedure in frozen sections of skeletal muscle biopsies as a valid indicator of malignant hyperthermia susceptibility.

Animals↗
PubMed1987

Neurofibrillary tangles in association with congenital hydrocephalus.

Morphologic study of the brain from a retarded patient with progressive congenital hydrocephalus revealed widespread neurofibrillary tangles in the cerebral cortex and the brain stem. Senile plaques were not found. The tangles consisted of paired helical filaments. Scanning electron microscopic energy spectrometric analysis failed to demonstrate abnormal accumulation of aluminum or other elements in the involved neurons.For the first time the association of congenital hydrocephalus and neurofibrillary tangles is reported. This observation is consistent with the hypothesis of interference with axonal transport of neurofilaments as a common pathogenetic mechanism in the genesis of neurofibrillary tangles in human brains.

Adult↗
PubMed1986

Tropism of sheep lentiviruses for monocytes: susceptibility to infection and virus gene expression increase during maturation of monocytes to macrophages.

Visna lentiviruses have a natural tropism for cells of the macrophage lineage of sheep and goats, but virus replication in these cells in vivo is restricted so that only small quantities of virus are produced. One restricting factor suggested in previous studies is that virus replication is dependent on the maturity of the cells: the more mature the cell, the less restrictive the replication of the virus. Since monocytes in peripheral blood are precursors of macrophages, we investigated the effect of cell maturation on virus replication under limited control conditions in vitro by inoculating blood leukocytes with virus and retarding the maturation of monocytes to macrophages during cultivation in serum-free medium. Using enzyme markers that identified the cells in their resting monocytic stage (peroxidase) and mature macrophage stage (acid phosphatase) along with quantitative in situ hybridization and immunocytochemistry with viral reagents to trace the efficiency of virus replication, we correlated virus replication with cell maturation. Only a few monocytes were susceptible to infection, and virus replication did not extend beyond a low level of transcription of viral RNA. In the acid phosphatase-positive, maturing macrophage, susceptibility of the cells to infection was increased and virus replication was greatly amplified to the level of translation of viral polypeptides. However, virus maturation was delayed by 3 days until further cell maturation had occurred. Thus, the entire life cycle of the virus, from its attachment to the target cell to its maturation in the cell, was dependent on the level of maturation/differentiation of the monocytic cell.

Animals↗
PubMed1986

Types of myofibers parasitized in experimentally induced infections with Sarcocystis cruzi and Sarcocystis capracanis.

Eighteen calves were orally inoculated with either 200,000 or 225,000 sporocysts of Sarcocystis cruzi. Eight goats were orally inoculated with 20,000 sporocysts of S capracanis. Calves and goats were euthanatized at various times after inoculation, and portions of their right and left biceps femoris, right and left longissimus dorsi, myocardium, and tongue were frozen at -150 C in precooled isopentane and stored at -70 C. Frozen sections of these muscles were stained with hematoxylin and eosin, modified Gomori's trichrome, nonspecific esterase, diphosphopyridine nucleotide tetrazolium reductase, and adenosinetriphosphatase at pH 10.4 and 4.6. Muscle from the same locations was fixed in 10% neutral buffered formalin, processed for paraffin embedding, sectioned, and stained with hematoxylin and eosin. Microscopic examination of both calf and goat tissue indicated that both type I and type II muscle fibers were equally infected and that infected myofibers showed no apparent damage other than displacement by sarcocysts. Occasionally, muscle fibers within the muscle spindles contained sarcocysts.

Animals↗
PubMed1985

Crystalline encephalopathy: cerebral immunoprotein deposits and isolated angiitis.

Brain biopsy specimens from a 38-year-old woman with adversive seizures and bifrontal mass lesions evident on computed tomographic scans showed extracellular and intracellular deposits of crystallized proteins. These were morphologically identical to the immunoglobulin crystals seen in reactive or neoplastic plasma cells and by peroxidase-antiperoxidase methods were found to contain polyclonal immunoglobulins. In addition, severe angiitis of the intracerebral blood vessels was present.

Adult↗
PubMed1985

Tolerance of peripheral nerve to intraoperative radiotherapy (IORT): clinical and experimental studies.

In our clinical experience combining wide excision and intraoperative radiotherapy (IORT), five patients have developed clinical signs of lumbosacral or sciatic neuropathy within 9 months of receiving IORT to a dose of 20-25 Gy. Three patients showed recovery of nerve function over several months while two patients have shown no recovery and have near complete loss of extremity function. In an attempt to investigate this clinical observation further, the lumbosacral plexus and sciatic nerve of American foxhounds were surgically exposed and received a single dose of IORT ranging from 20-75 Gy. An approximate linear relationship between radiation dose and time to onset of hind limb paresis is found with 19 of 21 irradiated dogs showing clinical signs of nerve injury within an interval of 1-19 months. No recovery of nerve function is seen in these dogs. Histological study of the irradiated nerves demonstrates a loss of nerve fibers, particularly those of the large myelinated type without evidence of vascular occlusion or thrombosis. These studies suggest that peripheral nerve may be a dose-limiting normal tissue in clinical studies of IORT.

Adult↗
PubMed1985

Immunoperoxidase study of primary central nervous system lymphomas.

Primary central nervous system (CNS) lymphomas have been perceived as CNS counterparts of systemic non-Hodgkin's lymphomas (NHL). Their pathogenesis in respect to the cell of origin, however, has been controversial. A highly sensitive and specific immunoperoxidase method for cytoplasmic immunoglobulins (CIg) using anti-kappa and anti-lambda light-chain antisera in addition to antibodies against IgM, IgG, IgA and IgE heavy chains and J-chain was performed on 27 surgically removed and histologically confirmed primary CNS lymphomas. In order to increase the sensitivity, slides were treated with trypsin to expose the various CIg components. Results indicated that the majority of CNS lymphomas (20 cases or 74.01 percent) were negative for monoclonal CIg. Only four cases (14.81 percent) were definitely positive for CIg with monoclonal staining pattern. Results of the remaining three cases were inconclusive. Among those four cases with positive CIg, three were histologically identified as immunoblastic sarcomas according to the Luke-Collins classification. It is concluded that, in contrast to systemic NHL, primary CNS lymphomas are mostly negative for monoclonal CIg. Whether these CIg negative neoplasms are T and/or null cells in nature or whether they represent an unidentified group of neoplasms is not clear at the present.

Adult↗