Menkes disease: a Golgi and electron microscopic study of the cerebellar cortex.
Identifieur interne : 002441 ( Main/Corpus ); précédent : 002440; suivant : 002442Menkes disease: a Golgi and electron microscopic study of the cerebellar cortex.
Auteurs : O. Robain ; P. Aubourg ; M C Routon ; O. Dulac ; G. PonsotSource :
- Clinical neuropathology [ 0722-5091 ]
English descriptors
- KwdEn :
- MESH :
- pathology : Brain Diseases, Metabolic, Cerebellar Cortex, Menkes Kinky Hair Syndrome, Purkinje Cells.
- ultrastructure : Cerebellar Cortex, Purkinje Cells.
- Child, Preschool, Humans, Male, Microscopy, Electron.
Abstract
A neuropathological study of a case of Menkes disease is reported, illustrating the involvement of different types of neuronal cells. The cerebellum showed the most striking abnormalities: severe lack of internal granule cells. Purkinje cells with weeping willow pattern, numerous segmental enlargements of dendritic trunks and secondary branches, and presence of numerous eosinphilic spherical bodies in the molecular layer were the most conspicuous features. Using electron microscopy, the dendritic enlargements were observed to be made of both proliferated and enlarged mitochondria, and of saccules of smooth endoplasmic reticulum. The spheroid bodies in the molecular layer were mainly made of concentric lamellar structures which seemed to be proliferated smooth endoplasmic reticulum. The relationship between these morphological abnormalities and the metabolic disorder of Menkes disease is discussed.
PubMed: 3390973
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Ponsot</name></author></titleStmt><publicationStmt><idno type="wicri:source">PubMed</idno><date when="1988">1988 Mar-Apr</date><idno type="RBID">pubmed:3390973</idno><idno type="pmid">3390973</idno><idno type="wicri:Area/Main/Corpus">002441</idno><idno type="wicri:explorRef" wicri:stream="Main" wicri:step="Corpus" wicri:corpus="PubMed">002441</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en">Menkes disease: a Golgi and electron microscopic study of the cerebellar cortex.</title><author><name sortKey="Robain, O" sort="Robain, O" uniqKey="Robain O" first="O" last="Robain">O. Robain</name><affiliation><nlm:affiliation>INSERM U-29, Paris, France.</nlm:affiliation></affiliation></author><author><name sortKey="Aubourg, P" sort="Aubourg, P" uniqKey="Aubourg P" first="P" last="Aubourg">P. Aubourg</name></author><author><name sortKey="Routon, M C" sort="Routon, M C" uniqKey="Routon M" first="M C" last="Routon">M C Routon</name></author><author><name sortKey="Dulac, O" sort="Dulac, O" uniqKey="Dulac O" first="O" last="Dulac">O. Dulac</name></author><author><name sortKey="Ponsot, G" sort="Ponsot, G" uniqKey="Ponsot G" first="G" last="Ponsot">G. Ponsot</name></author></analytic><series><title level="j">Clinical neuropathology</title><idno type="ISSN">0722-5091</idno></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Brain Diseases, Metabolic (pathology)</term><term>Cerebellar Cortex (pathology)</term><term>Cerebellar Cortex (ultrastructure)</term><term>Child, Preschool (MeSH)</term><term>Humans (MeSH)</term><term>Male (MeSH)</term><term>Menkes Kinky Hair Syndrome (pathology)</term><term>Microscopy, Electron (MeSH)</term><term>Purkinje Cells (pathology)</term><term>Purkinje Cells (ultrastructure)</term></keywords><keywords scheme="MESH" qualifier="pathology" xml:lang="en"><term>Brain Diseases, Metabolic</term><term>Cerebellar Cortex</term><term>Menkes Kinky Hair Syndrome</term><term>Purkinje Cells</term></keywords><keywords scheme="MESH" qualifier="ultrastructure" xml:lang="en"><term>Cerebellar Cortex</term><term>Purkinje Cells</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Child, Preschool</term><term>Humans</term><term>Male</term><term>Microscopy, Electron</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">A neuropathological study of a case of Menkes disease is reported, illustrating the involvement of different types of neuronal cells. The cerebellum showed the most striking abnormalities: severe lack of internal granule cells. Purkinje cells with weeping willow pattern, numerous segmental enlargements of dendritic trunks and secondary branches, and presence of numerous eosinphilic spherical bodies in the molecular layer were the most conspicuous features. Using electron microscopy, the dendritic enlargements were observed to be made of both proliferated and enlarged mitochondria, and of saccules of smooth endoplasmic reticulum. The spheroid bodies in the molecular layer were mainly made of concentric lamellar structures which seemed to be proliferated smooth endoplasmic reticulum. The relationship between these morphological abnormalities and the metabolic disorder of Menkes disease is discussed.</div></front></TEI><pubmed><MedlineCitation Status="MEDLINE" Owner="NLM"><PMID Version="1">3390973</PMID><DateCompleted><Year>1988</Year><Month>08</Month><Day>12</Day></DateCompleted><DateRevised><Year>2004</Year><Month>11</Month><Day>17</Day></DateRevised><Article PubModel="Print"><Journal><ISSN IssnType="Print">0722-5091</ISSN><JournalIssue CitedMedium="Print"><Volume>7</Volume><Issue>2</Issue><PubDate><MedlineDate>1988 Mar-Apr</MedlineDate></PubDate></JournalIssue><Title>Clinical neuropathology</Title><ISOAbbreviation>Clin Neuropathol</ISOAbbreviation></Journal><ArticleTitle>Menkes disease: a Golgi and electron microscopic study of the cerebellar cortex.</ArticleTitle><Pagination><MedlinePgn>47-52</MedlinePgn></Pagination><Abstract><AbstractText>A neuropathological study of a case of Menkes disease is reported, illustrating the involvement of different types of neuronal cells. The cerebellum showed the most striking abnormalities: severe lack of internal granule cells. Purkinje cells with weeping willow pattern, numerous segmental enlargements of dendritic trunks and secondary branches, and presence of numerous eosinphilic spherical bodies in the molecular layer were the most conspicuous features. Using electron microscopy, the dendritic enlargements were observed to be made of both proliferated and enlarged mitochondria, and of saccules of smooth endoplasmic reticulum. The spheroid bodies in the molecular layer were mainly made of concentric lamellar structures which seemed to be proliferated smooth endoplasmic reticulum. The relationship between these morphological abnormalities and the metabolic disorder of Menkes disease is discussed.</AbstractText></Abstract><AuthorList CompleteYN="Y"><Author ValidYN="Y"><LastName>Robain</LastName><ForeName>O</ForeName><Initials>O</Initials><AffiliationInfo><Affiliation>INSERM U-29, Paris, France.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Aubourg</LastName><ForeName>P</ForeName><Initials>P</Initials></Author><Author ValidYN="Y"><LastName>Routon</LastName><ForeName>M C</ForeName><Initials>MC</Initials></Author><Author ValidYN="Y"><LastName>Dulac</LastName><ForeName>O</ForeName><Initials>O</Initials></Author><Author ValidYN="Y"><LastName>Ponsot</LastName><ForeName>G</ForeName><Initials>G</Initials></Author></AuthorList><Language>eng</Language><PublicationTypeList><PublicationType UI="D002363">Case Reports</PublicationType><PublicationType UI="D016428">Journal Article</PublicationType></PublicationTypeList></Article><MedlineJournalInfo><Country>Germany</Country><MedlineTA>Clin Neuropathol</MedlineTA><NlmUniqueID>8214420</NlmUniqueID><ISSNLinking>0722-5091</ISSNLinking></MedlineJournalInfo><CitationSubset>IM</CitationSubset><MeshHeadingList><MeshHeading><DescriptorName UI="D001928" MajorTopicYN="N">Brain Diseases, Metabolic</DescriptorName><QualifierName UI="Q000473" MajorTopicYN="Y">pathology</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D002525" MajorTopicYN="N">Cerebellar Cortex</DescriptorName><QualifierName UI="Q000473" MajorTopicYN="Y">pathology</QualifierName><QualifierName UI="Q000648" MajorTopicYN="N">ultrastructure</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D002675" MajorTopicYN="N">Child, Preschool</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D006801" MajorTopicYN="N">Humans</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D008297" MajorTopicYN="N">Male</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D007706" MajorTopicYN="N">Menkes Kinky Hair Syndrome</DescriptorName><QualifierName UI="Q000473" MajorTopicYN="Y">pathology</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D008854" MajorTopicYN="N">Microscopy, Electron</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D011689" MajorTopicYN="N">Purkinje Cells</DescriptorName><QualifierName UI="Q000473" MajorTopicYN="N">pathology</QualifierName><QualifierName UI="Q000648" MajorTopicYN="N">ultrastructure</QualifierName></MeshHeading></MeshHeadingList></MedlineCitation><PubmedData><History><PubMedPubDate PubStatus="pubmed"><Year>1988</Year><Month>3</Month><Day>1</Day></PubMedPubDate><PubMedPubDate PubStatus="medline"><Year>1988</Year><Month>3</Month><Day>1</Day><Hour>0</Hour><Minute>1</Minute></PubMedPubDate><PubMedPubDate PubStatus="entrez"><Year>1988</Year><Month>3</Month><Day>1</Day><Hour>0</Hour><Minute>0</Minute></PubMedPubDate></History><PublicationStatus>ppublish</PublicationStatus><ArticleIdList><ArticleId IdType="pubmed">3390973</ArticleId></ArticleIdList></PubmedData></pubmed></record>Pour manipuler ce document sous Unix (Dilib)
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