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At least 271 records · Page 15Linked to original sources
PubMed1991

Regional distribution of somatostatin receptor binding and modulation of adenylyl cyclase activity in Alzheimer's disease brain.

We have previously reported a reduction in the inhibitory effect of somatostatin on adenylyl cyclase activity in the superior temporal cortex of a group of Alzheimer's disease cases, compared to a group of matched controls. In the present study, the levels of high affinity 125I-Tyr11-somatostatin-14 binding, its modulation by guanine nucleotides and the effects of somatostatin on adenylyl cyclase activity have been measured in preparations of frontal cortex, hippocampus, caudate nucleus and cerebellum from the same patient and control groups. A significant reduction in 125I-Tyr11-somatostatin-14 binding was observed in the frontal cortex, but not other regions, of the Alzheimer's disease group, compared with control values. The profiles of inhibition of specific 125I-Tyr11-somatostatin-14 binding by Gpp(NH)p were similar in all regions in both groups. No significant differences in basal, forskolin-stimulated, or somatostatin and neuropeptide Y inhibitions of adenylyl cyclase activity were found between the two groups. The pattern of change of somatostatin binding in the Alzheimer's disease cases observed in the present study differs from the reported pattern of loss of somatostatin neurons and may be secondary to the degeneration of somatostatin receptor-bearing cholinergic afferents arising from the nucleus basalis. The results of this study indicate that impaired somatostatin modulation of adenylyl cyclase is not a global phenomenon in Alzheimer's disease brain and also that there are no major disruptions of somatostatin receptor-G-protein coupling or of adenylyl cyclase catalytic activity in this disorder.

Adenylyl Cyclases↗
PubMed1989

Alpha B-crystallin is expressed in non-lenticular tissues and accumulates in Alexander's disease brain.

Rosenthal fibers (RFs) are abnormal inclusions within astrocytes, characteristic of Alexander's disease. We have previously isolated a 22 kd protein component of RFs from Alexander's disease brain. By Western blotting, we detected its equivalent in several rat organs, with the highest level in heart, and in a human astrocytoma cell line (U-373MG). A cDNA library established from U-373MG was screened with an anti-RF protein antibody. A partial cDNA clone encoding the lens protein alpha B-crystallin was isolated. The anti-RF protein antibodies react with lens alpha B-crystallin. Furthermore, the distribution of alpha B-crystallin mRNA in rat organs is consistent with the Western blots. Therefore, alpha B-crystallin is not lens-specific and it can accumulate in large amounts in astrocytes in pathological conditions.

Amino Acid Sequence↗
PubMed2005

The function of the neuronal proteins Shc and huntingtin in stem cells and neurons: pharmacologic exploitation for human brain diseases.

The identification of intracellular molecules and soluble factors that are important for neuronal differentiation and survival are of critical importance for development of therapeutic strategies for brain diseases. First, the activity of these factors/molecules may be enhanced in vivo in the attempt to induce proper neuronal differentiation and integration of the resident stem cells. Second, these factors may be applied ex vivo to increase the recovery of neurons from stem cells. Third, for those intracellular molecules that play crucial roles in neuronal survival, identification of their downstream targets may give us the chance to develop drug screening assays that use these targets for therapeutic purposes. In recent years, it has become evident that intracellular signaling processes are critical mediators of the responses of neural stem cells and neurons to growth factors. Analysis of the mechanisms of signal transduction has led to the striking finding that a handful of conserved signaling pathways appear to be used in different combinations to specify a wide variety of tissues or cells. This review will focus on the mechanisms by which specific molecules control the transition from proliferation to differentiation of neural progenitor cells and the subsequent survival of postmitotic neurons; it also discusses how this knowledge may be exploited to increase the potential efficacy of stem cell replacement in the damaged brain.

Adaptor Proteins, Signal Transducing↗
PubMed1988

Receptors in human brain diseases: a use for receptor autoradiography in neuropathology.

The application of the technique of in vitro receptor autoradiography to the study of neurotransmitter receptor alterations associated with human brain diseases is reviewed. Focus is made on receptor alterations in Parkinson's disease and Senile Dementia. Different types of modifications have been found. The most important are 1) receptor changes related to a specific neuronal loss; 2) receptor regulation associated with modifications of presynaptic input; 3) receptor changes which cannot directly be related to neuronal loss or presynaptic changes. The possible value as a diagnostic tool and the utility of receptor autoradiography in neuropathology is discussed.

Autoradiography↗
PubMed1984

Brain disease and psychiatric illness: when should a psychiatrist order a CAT scan?

Because psychiatric symptoms are frequently the earliest signs of CNS pathology, the author outlines the structural brain diseases that may underlie several psychiatric syndromes and describes the computerized axial tomographic (CAT) scan findings associated with those diseases. On the basis of an awareness of these conditions and a cost-benefit analysis, the following indications for CAT scanning of psychiatric patients are proposed: 1) confusion and/or dementia of unknown cause, 2) first episode of a psychosis of unknown etiology, 3) movement disorder of unknown etiology, 4) anorexia nervosa, 5) prolonged catatonia, and 6) first episode of major affective disorder or personality change after age 50.

Adult↗
PubMed2001

A child with muscle-eye-brain disease. Ophthalmological and neurological characteristics.

PURPOSE: To describe a child with Muscle-Eye-Brain disease (MEB), one of three types of congenital muscular dystrophy associated with ocular abnormalities. METHODS: Case report. RESULTS: The child showed severe visual impairment due to progressive myopia and retinal degeneration, a pachygyria-type of migration disorder of the brain with a nodular cortical surface, i.e. cobblestone cortex, as well as muscular weakness and severe mental retardation. CONCLUSION: Ophthalmological assessments are important to help to diagnose and follow children with congenital muscular dystrophy.

Abnormalities, Multiple↗
PubMed2003

Music and the brain. Lessons from brain diseases and some reflections on the "emotional" brain.

Studies are reviewed from the perspective of a neurologist and epileptologist interested in "music and the brain." At the neurocognitive level, deficits in pitch discrimination of patients with brain lesions and those during the intracarotid amobarbital test are outlined, because they show that the temporal lobe and, in particular, the right acoustic cortex are crucial. Hallucinations of music during epileptic seizures as well as the analysis of musicogenic epilepsy point to the same gross localization and lateralization. At the esthetic level, music theoretical concepts on the consonance-dissonance dichotomy and related EEG examinations are reported, which illustrate the importance of mesiolimbic temporal lobe structures for the pleasure that we might experience when listening to music. The complex interaction of many neuronal circuits and assemblies of both hemispheres in musical perception and performance is illustrated by musical analysis of a recording by an organ player who experienced a right temporal lobe seizure. This analysis revealed that the seizure-induced errors of the left hand were compensated with the right hand in a musically meaningful way.

Emotions↗
PubMed1991

Association of amyloid P component with complement proteins in neurologically diseased brain tissue.

Amyloid P component (AP) was detected in a number of lesions in human brain associated with chronic degenerative disease. These lesions included diffuse and consolidated amyloid deposits, cerebral amyloid angiopathy, neurofibrillary tangles, neuropil threads and complement activated oligodendroglia. The staining for AP generally paralleled that for complement proteins C4d and C3d, suggesting that AP may serve as an adjuvant for phagocytosis.

Alzheimer Disease↗
PubMed1995

Functional magnetic resonance imaging. Application to degenerative brain disease and hydrocephalus.

Magnetic resonance (MR) imaging, in addition to its excellent depiction of neuroanatomy, is being developed as a major technique for functional imaging of cerebrospinal fluid motion and for measurement of velocity, strain, and diffusional processes within the brain parenchyma. Functional MR imaging studies are revealing basic physiology of blood flow interactions with CSF motion and dynamic processes of brain parenchyma. Normal pressure hydrocephalus and degenerative brain disease are current focuses of functional MR imaging studies.

Brain↗
PubMed1991

Neuropathological findings in muscle-eye-brain disease (MEB-D). Neuropathological delineation of MEB-D from congenital muscular dystrophy of the Fukuyama type.

Congenital muscular dystrophy (CMD) associated with cerebro-ocular dysplasia named muscle-eye-brain disease (MEB-D) is described in two sisters. Progressive hypotonia, mental retardation and severe visual failure appeared immediately after birth. Pathological examination demonstrated muscular dystrophy, hydrocephalus, type II lissencephaly and defective eye development of foetal origin. The great similarity of the clinical and neuropathological picture of both sisters is in agreement with an autosomal recessive inheritance. Neuropathological distinction between Fukuyama-CMD and MEB-D is a more severe and earlier cerebral developmental defect and the association with ocular dysplasia in MEB-D.

Brain↗
PubMed1987

Ubiquitin is detected in neurofibrillary tangles and senile plaque neurites of Alzheimer disease brains.

Neurofibrillary tangles (NFT) and neurites associated with senile plaques (SP) in Alzheimer disease-affected brain tissues were specifically immunostained with affinity-purified antibody preparations directed against ubiquitin. In addition, a class of neurites seen in brain regions containing NFT and SP were also specifically stained. Cross-reactivity of the ubiquitin antisera for tau protein, neurofilament proteins, and high molecular weight microtubule-associated proteins (MAPs) were ruled out by (i) the inability of the ubiquitin antisera to stain these proteins in immunoblotting experiments and (ii) the inability of tau, neurofilament, and MAP preparations, when preincubated with the ubiquitin antisera, to inhibit the selective neurofibrillar staining observed. Our results are consistent with the suggestion that ubiquitin is covalently associated with the insoluble neurofibrillary material of NFT and SP. We propose that the ubiquitin-mediated degradative pathway may be ineffective in removing these fibrillar structures in Alzheimer disease brain.

Aged↗
PubMedDate not supplied

Isolation of a phosphorylated soluble tau fraction from Alzheimer's disease brain.

Modified forms of tau proteins are major components of the paired helical filaments (PHFs) present in Alzheimer brains. In this study, tau from cytosolic samples obtained from normal and Alzheimer disease brains were fractionated by iron-chelated affinity chromatography (ICAC) to discriminate between isoforms phosphorylated to different extents using an stepwise pH gradient. Immunoblot analysis of the different fractions using antibody Tau-1 (recognizing an unphosphorylated epitope in tau and in PHF-tau after dephosphorylation) and antibody SMI 31 (recognizing a phosphorylated epitope in PHF-tau) have been carried out. Phosphorylated tau species (Tau 1-nonreactive and SMI 31-reactive) are only isolated from the Alzheimer samples at pH = 8.5. These tau species although having other Ser/Thr-Pro motifs susceptible of phosphorylation by proline-directed protein kinases are not further phosphorylated in vitro by MAP2 kinase whereas the fraction isolated at pH 7.0, which contains underphosphorylated tau species, is phosphorylated. Thus, soluble tau species phosphorylated both at the sites constituting the Tau-1 and the SMI 31 epitopes are present in Alzheimer but not in normal brain cytosol and can be isolated by ICAC. These modifications may be a prerequisite for PHF formation.

Aged↗
PubMed1994

Brain disease and molecular analysis in myotonic dystrophy.

Abnormal amplification of a CTG repeat on chromosome 19 is the molecular basis of myotonic dystrophy (DM). Expansion of the repeat has been correlated with severity of several clinical features of the disease. We performed extensive cognitive testing, cerebral magnetic resonance imaging (MRI) and a molecular analysis in 28 cases of DM to determine the relationship between the molecular defect and brain disease. Performance in two or more cognitive tests was pathological in 10 cases. Fourteen patients had subcortical white matter lesions on MRI, 14 had cerebral atrophy. Amplification of the CTG repeat showed a strong correlation with cognitive test deficits when exceeding a length of over 1000 trinucleotides. MRI lesions were associated with impaired psychometric performance, but MRI and molecular findings were only weakly related. Disease duration influenced the appearance and amount of white matter lesions on MRI. Quantification of CTG repeat size may allow an early estimate on the probability of brain involvement in DM; cognitive dysfunction is associated with white matter lesions and cerebral atrophy later on in the course.

Adult↗
PubMed1997

Iron deposits in multiple sclerosis and Alzheimer's disease brains.

Iron may contribute to the pathogenesis of neurological diseases by promoting oxidative damage. The localization of iron in multiple sclerosis (MS) and Alzheimer's disease (AD) brains was investigated to further the understanding of its pathogenic role in these disease states. Earlier studies, utilizing a standard Perls' stain, yielded conflicting reports regarding the distribution of iron deposits in MS brains, and a previous study on AD brains utilized a diaminobenzidine (DAB) enhanced version of this stain. In the present study, a modified version of the DAB-enhanced stain was used; it utilizes sodium borohydride, proteinase K, Triton X-100 and xylenes to increase the accessibility of tissue iron to histochemical reagents. This modified method can reveal iron deposits that are missed by the Perls' or DAB-enhanced Perls' stains. In addition to its normal deposition in oligodendrocytes and myelin, iron was detected in reactive microglia, ameboid microglia and macrophages in MS brains. In AD brains, three types of plaques were stained: dense core, clear core and amorphous plaques. Punctate staining was also observed in neurons in the corticies of AD brains. The structure accounting for punctate labeling may be damaged mitochondria, lipofuscin or amyloid deposits. Dense core plaques, clear plaques and punctate labeling were not detected in the previous AD study which utilized only the DAB-enhanced Perls' stain. The labeling of these additional structures illustrates the benefit of the modified method. In summary, the localization of iron deposition in MS and AD brains indicates potential sites where iron could promote oxidative damage in these disease states.

Alzheimer Disease↗