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J Gheuens

Publications and source records attributed to J Gheuens.

At least 37 records · Page 2Linked to original sources
PubMed1989

Exclusion analysis of Charcot-Marie-Tooth neuropathy (CMT1) with chromosome 1p markers.

We previously described a large five-generation family with autosomal dominant inheritance of hereditary motor and sensory neuropathy type I, or Charcot-Marie-Tooth disease (CMT1). The genetic defect in this family was not linked to the Duffy blood group. We investigated the possibility of a disease locus on the short arm of chromosome 1 using 12 anonymous DNA markers. Two markers, D1S2 and D1S22, showed positive linkage, suggesting the existence of a CMT1 locus on 1p. D1S2 and D1S22 are clustered in the 1p31----p22 region. However, multipoint linkage analysis, including additional DNA markers from this chromosome region, excluded a possible CMT1 locus in this part of chromosome 1.

Charcot-Marie-Tooth Disease↗
PubMed1989

Absence of genetic linkage of Charcot-Marie-Tooth disease (HMSN Ia) with chromosome 1 gene markers.

We previously reported a large Charcot-Marie-Tooth family not linked to the Duffy blood group marker, supporting the existence of genetic heterogeneity in this neuropathy. In order to investigate the possibility of another disease locus on chromosome 1, we analyzed this family further, using DNA polymorphisms of 6 genes. Absence of linkage makes a second disease locus on chromosome 1 unlikely.

Charcot-Marie-Tooth Disease↗
PubMed1989

Lazarus sign and extensor posturing in a brain-dead patient. Case report.

A man was declared brain dead after having sustained a gunshot wound to the head. All clinical criteria for the diagnosis of brain death were met. The electroencephalogram was isoelectric, and four-vessel angiography demonstrated the absence of cerebral blood flow. However, stereotypic spontaneous movements were observed which persisted for several hours. The possible mechanism is discussed and a short review of the literature is given.

Brain Death↗
PubMed1989

Localization of the mutation in an extended family with Charcot-Marie-Tooth neuropathy (HMSN I).

Hereditary motor and sensory neuropathy type I (HMSN I) or Charcot-Marie-Tooth (CMT) disease is an autosomal dominant peripheral neuropathy. In some CMT families linkage has been reported with either the Duffy blood group or the APOA2 gene, both located on chromosome 1q. More recently, linkage has been found in six CMT families with two chromosome 17p markers. We extensively analyzed a multi-generation Charcot-Marie-Tooth family by using molecular genetic techniques in order to localize the CMT gene defect. First, we constructed a continuous linkage group of 11 chromosome 1 markers and definitely excluded chromosome 1 as the site of mutation. Second, we analyzed the family for linkage with chromosome 17. The two-point lod scores obtained with D17S58 and D17S71 proved that this Charcot-Marie-Tooth family is linked to chromosome 17. Moreover, multipoint linkage results indicated that the mutation is most likely located on the chromosome 17p arm, distal of D17S71.

Charcot-Marie-Tooth Disease↗
PubMed1988

The Duffy blood group is linked to the alpha-spectrin locus in a large pedigree with autosomal dominant inheritance of Charcot-Marie-Tooth disease type 1.

The alpha-spectrin locus (SPTA) on chromosome 1 maps to 1q22-q25 and alpha-spectrin specific probes detect restriction fragment length polymorphisms (RFLPs) with the endonucleases MspI and PvuII. The Duffy blood group (FY) has been mapped to the 1p21-q23 region. We found positive linkage between the alpha-spectrin and the Duffy loci with a maximal Lod score of 3.81 at theta = 0.0 using the computer program MLINK. This indicates that both loci are very closely linked and probably localized to 1q22-q23.

Blood Group Antigens↗
PubMed1988

Gamma-enolase and glial fibrillary acidic protein in nervous system tumors. An immunohistochemical study using specific monoclonal antibodies.

A large series of central and peripheral nervous system tumors was studied for the presence of glial fibrillary acidic protein (GFAP) and gamma-enolase (neuron-specific enolase, NSE), using specific monoclonal antibodies (mAbs). Occurrence in and specificity of GFAP to glial and mixed tumors was confirmed and depended on the malignancy grade and features such as meningeal invasion. Using a well-characterized mAb, gamma-enolase was demonstrated in neuronal, as well as in a whole range of non-neuronal tumors. This lack of specificity of gamma-enolase prohibits its use as an exclusive neuronal marker. Nevertheless quantization or comparison with other types of enolases could still prove to be useful in well-defined situations. The advantages inherent to mAbs and a highly sensitive detection system turn GFAP stainings into a specific and readily reproducible technique.

Antibodies, Monoclonal↗
PubMed1988

High resolution nuclear magnetic resonance imaging of the spinal cord in experimental demyelinating disease.

Chronic recurrent experimental allergic encephalomyelitis was induced in a strain 13 guinea pig by inoculation of isologous spinal cord homogenate. The spinal cord was obtained after perfusion with 4% paraformaldehyde and examined with nuclear magnetic resonance (NMR) imaging. Proton NMR spin echo images (repetition time: 3 s; echo times: 20 and 60 ms) were obtained from intact, isolated spinal cord in a 4.7 Tesla, 50 mm bore magnet. The slice thickness of the images was 380 microns and the inplane resolution was 40 X 40 microns. The images showed superficial areas of low signal intensity in the lateroventral regions of the white matter, in some instances with a seam of higher signal intensity. Neuropathologically, these abnormalities corresponded exactly to areas of demyelination. Control images did not show these abnormalities. The present high resolution imaging allowed a correlation between demyelination and abnormal NMR signals in a small laboratory animal with an inflammatory demyelinating disease.

Animals↗
PubMed1988

Absence of linkage with the Duffy blood group in a family with Charcot-Marie-Tooth neuropathy.

We report a large Belgian family with Charcot-Marie-Tooth disease (CMT) or hereditary motor and sensory neuropathy type I (HMSN-I). The pedigree consists of 5 generations with 350 family members comprising 42 patients. The disease is transmitted according to an autosomal dominant inheritance pattern. Several HMSN-I families have been reported to be closely linked to the Duffy blood group marker on chromosome 1. These families were designated HMSN-Ib families, opposed to the HMSN-Ia families which do not show evidence for such a linkage. Therefore we examined our family for the Duffy linkage relationship. We found no evidence for a strong linkage of the disease to the Duffy blood group locus, indicating that this family is of genetic subtype Ia.

Blood Group Antigens↗
PubMed1987

Value of glial fibrillary acidic protein determination in amniotic fluid for prenatal diagnosis of neural tube defects.

Glial fibrillary acidic protein (GFAp), an intracellular protein specific to astrocytes of the central nervous system, was determined by 2-site immunoradiometric assay in amniotic fluid from 78 pregnancies with a normal, and 100 with an abnormal outcome. GFAp was not detectable in any of the normal pregnancies, but there were measurable, and so raised, levels in 23 out of 25 cases of anencephaly and 4 out of 7 cases of spina bifida, which therefore allowed prenatal diagnosis. GFAp was also increased in 4 of 6 cases of fetal intrauterine death, but not in other congenital malformations associated with elevated alphafetoprotein levels or abnormal acetylcholinesterase banding pattern, such as exomphalos, other gastrointestinal malformations or renal abnormalities. GFAp is therefore specific for diagnosing open neural tube defects. The determination of GFAp in amniotic fluid was slightly less efficient overall than AFP for the prenatal diagnosis of neural tube defects, but can be a useful ancillary test and has the advantage of specificity.

Acetylcholinesterase↗
PubMed1987

Expression of glial fibrillary acidic protein in rat C6 glioma relates to vimentin and is independent of cell-cell contact.

Glial fibrillary acidic protein (GFAP) was induced in rat C6 glioma cells grown in M199 and HAM F10 media by addition of 1 mM dibutyryl cyclic AMP. The amount of GFAP per cell increased 7- and 33-fold in M199 and HAM F10 media, respectively. GFAP could be induced in each phase of the cell culture except for the lag phase, where GFAP synthesis was delayed until the onset of the logarithmic growth. The induction took place under conditions where the total protein content of the cell decreased. Measurement of the amount of vimentin indicated that GFAP was induced under conditions of low vimentin concentration. Our results do not support the hypothesis that GFAP induction depends on cell-cell contact or cell proliferation. They indicate a shift from vimentin to GFAP synthesis by an as yet unknown mechanism.

Animals↗
PubMed1987

Human gamma gamma-enolase: two-site immunoradiometric assay with a single monoclonal antibody.

A monoclonal antibody (mAb), termed BBS/NC/VI-H14 (H14), that reacts with the human enzyme gamma gamma-enolase was prepared. It was directed against the gamma-subunit and did not cross-react with the alpha- or beta-subunit. The mAb H14 can be used for quantitative determination of gamma gamma-enolase in a two-site immunoradiometric assay (two-site IRMA). It is also suitable for immunostaining formalin-fixed tissues. The specific identification of gamma gamma-enolase provided by the two-site IRMA with H14 is discussed in relation to the cellular distribution of this protein.

Animals↗
PubMed1985

The use of monoclonal antibodies in the study of the interaction between adrenal medullary cell membranes and chromaffin granules.

Simultaneous incubation of bovine adrenal medullary plasma membranes (PM) with chromaffin granules (CG) resulted in the release of the soluble granular content. The molecular mechanism of this process was studied with several monoclonal antibodies (mAb) raised against different plasma membrane components. Specific inhibition of the catecholamine secretion was obtained upon incubation with the monoclonal antibody UIA/NEU/VI B17. The corresponding antigen had an apparent molecular weight of 54000 Dalton. These results suggest a specific recognition between proteins located on the plasma membrane and chromaffin granule membrane, the interaction of which mediates exocytosis.

Adrenal Medulla↗
PubMed1984

Idiotypes and AIDS.

Explore the source record for details and available documents.

Acquired Immunodeficiency Syndrome↗
PubMed1984

Identification of several forms of the glial fibrillary acidic protein, or alpha-albumin, by a specific monoclonal antibody.

A monoclonal antibody (mAb), termed UIA/NEU/I/G1 (G1), that reacted with the astroglial marker glial fibrillary acidic protein (GFAP), or alpha-albumin, is described. It was directed against a structural determinant of GFAP. The G1 mAb could be used for quantitative determination of GFAP in two-site radiometric assays and for histoimmunological demonstration of GFAP. The G1 mAb reacted with the GFAP from rat as well as from man. The presence of several different molecular weight forms of GFAP in aqueous and detergent extracts from human brain was shown with the G1 mAb. The possible meaning of these forms is discussed.

Animals↗
PubMed1983

Antibody-mediated modification of encephalitis induced by hamster neurotropic measles virus.

The acute encephalopathy induced by hamster neurotropic (HNT) measles virus in BALB/c mice was abolished, and a subacute encephalitis was induced instead, when antibody to Edmonston (Ed) measles virus was administered three days after inoculation of HNT virus. Ascites fluid from 79XIC1, a monocolonal antibody to the hemaglutinin of strain Ed, had similar effects in vivo. However, other monoclonal antibodies to the hemagglutinin of Ed strain--79XVV17 and 80IIIB2--were ineffective in vivo at all doses tested. By viral neutralization, immunofluorescence, and radioimmunoprecipitation assays, it was shown that the hemagglutinin of HNT virus reacted with 79XIC1 antibody but lacked the epitopes recognized by 79XVV17 or 80IIIB2 antibody, a finding that explains their different reactivity in vivo. These observations also identified previously unrecognized antigenic differences between the hemagglutinin of Ed and HNT measles viruses.

Animals↗