Abstract：
:The colonic enteric nervous system was investigated in autopsy specimens from 12 patients with familial amyloidotic neuropathy (FAP) and 9 controls. The infiltration of amyloid deposits in the enteric nervous system was studied by double staining for amyloid and nerve elements. The myenteric plexus was immunostained for protein gene product (PGP) 9.5, vasoactive intestinal peptide (VIP), substance P and nitric oxide synthase (NOS). The immunostained nerve elements were quantified by computerised image analysis. Double staining revealed that there was no amyloid infiltration in the ganglia, or in the nerve fibres in the colonic enteric nervous system of FAP patients. The relative volume density of PGP 9.5-immunoreactive nerve fibres in both the circular and the longitudinal muscle layers in FAP patients did not differ significantly from that of controls. The relative volume density of VIP-immunoreactive nerve fibres in the circular muscle layer was significantly decreased in FAP patients compared with controls, but not in the longitudinal layer. The number of VIP-immunoreactive neurons/mm2 myenteric ganglia was significantly decreased in FAP patients. There were no statistical differences in the relative volume density for substance P- and NOS-immunoreactive nerve fibres between FAP patients and controls, nor was there any difference between FAP patients and controls regarding the number of NOS- and substance P-immunoreactive neurons/mm2 myenteric ganglia. It is concluded that the colonic enteric nervous system as a whole is intact and is not damaged by amyloid infiltration. The present observation of a reduction of VIP-immunoreactive nerve fibres and neurons in myenteric plexus of FAP patients might be one of the factors that contribute to the motility disorders seen in FAP patients.
journal_name
Acta Neuropatholjournal_title
Acta neuropathologicaauthors
Anan I,El-Salhy M,Ando Y,Forsgren S,Nyhlin N,Terazaki H,Sakashita N,Suhr OBdoi
10.1007/s004010051050subject
Has Abstractpub_date
1999-07-01 00:00:00pages
48-54issue
1eissn
0001-6322issn
1432-0533journal_volume
98pub_type
杂志文章abstract：:Multiple system atrophy (MSA) is characterized by the presence of distinctive glial cytoplasmic inclusions (GCIs) within oligodendrocytes that contain the neuronal protein alpha-synuclein (aSyn) and the oligodendroglia-specific phosphoprotein TPPP/p25α. However, the role of oligodendroglial aSyn and p25α in the format...
journal_title：Acta neuropathologica
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journal_title：Acta neuropathologica
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doi：10.1007/s004010051177
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journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00308480
更新日期：1992-01-01 00:00:00
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journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00299408
更新日期：1992-01-01 00:00:00
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pub_type： 杂志文章
doi：10.1007/BF00294292
更新日期：1993-01-01 00:00:00
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journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-002-0614-9
更新日期：2003-02-01 00:00:00
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journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00294460
更新日期：1995-01-01 00:00:00
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journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-014-1296-9
更新日期：2014-07-01 00:00:00
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journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s004010100411
更新日期：2001-12-01 00:00:00
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journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00294381
更新日期：1989-01-01 00:00:00
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journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00690959
更新日期：1985-01-01 00:00:00
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journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00690449
更新日期：1980-01-01 00:00:00
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journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00693778
更新日期：1987-01-01 00:00:00
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journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00293318
更新日期：1994-01-01 00:00:00
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journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-013-1224-4
更新日期：2014-05-01 00:00:00
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journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00688637
更新日期：1985-01-01 00:00:00
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journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00691427
更新日期：1986-01-01 00:00:00
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journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-012-1003-7
更新日期：2012-08-01 00:00:00
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journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-011-0802-6
更新日期：2011-03-01 00:00:00
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journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/pl00007408
更新日期：2000-01-01 00:00:00
abstract：:Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by the aggregation of ubiquitinated proteins in affected motor neurons. Recent studies have identified several new molecular constituents of ALS-linked cellular aggregates, including FUS, TDP-43, OPTN, UBQLN2 and the translational product...
journal_title：Acta neuropathologica
pub_type： 杂志文章,评审
doi：10.1007/s00401-013-1125-6
更新日期：2013-06-01 00:00:00
abstract：:A 1-year-old male chinchilla with a 2-week history of conjunctivitis suffered subsequently from neurological signs comprising seizures, disorientation, recumbency and apathy. After 3 weeks of progressive central nervous disease the animal was killed in view of the poor prognosis. A non-suppurative meningitis and polio...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-002-0597-6
更新日期：2002-12-01 00:00:00
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journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00687928
更新日期：1975-01-01 00:00:00
abstract：:Investigations of the existence and distribution of muscle spindles and tendon organs in the human voluntary anal sphincter muscle in 53 cases of autopsy material from both female and male persons (age: 27th week of gestation to 81 years) brought forward the following results: Similar to animals, muscle spindles are f...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00691860
更新日期：1984-01-01 00:00:00
abstract：:Inflammatory myopathy with abundant macrophages (IMAM) and dermatomyositis (DM) are considered to represent related disorders, since they share inflammatory infiltrates and skin alterations. In order to get more insight into these disorders, we addressed the cellular composition of the inflammatory infiltrates in musc...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-009-0570-8
更新日期：2009-12-01 00:00:00
abstract：:The colocalization of beta amyloid protein with the enzymes acetyl- and butyrylcholinesterase was assessed using immunocytochemistry for beta amyloid protein and a sensitive histochemical technique for cholinesterases. In non-demented aged and Alzheimer's disease brains, double-stained sections for cholinesterases and...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00334445
更新日期：1993-01-01 00:00:00
abstract：:Numbers of synapses were counted in columns of neuropil orientated at right angles to the pia in frontal and temporal regions from postmortem brains. In the frontal region of 28 patients aged 26-90 years there were 1.2 X 10(8) mm3 (SD 0.03 X 10(8], and in the temporal of 18 patients aged 16-90 years there were 1.4 X 1...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00684929
更新日期：1983-01-01 00:00:00
abstract：:Cerebrotendinous xanthomatosis (CTX), a rare autosomal-recessive lipid storage disease, has been well characterized clinically and biochemically, and recently also from the molecular biological aspect. However, only a very few publications deal with its neuropathology, and views on its pathogenesis vary. Based on a re...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00294324
更新日期：1995-01-01 00:00:00
