PMID- 8548127
OWN - NLM
STAT- MEDLINE
DCOM- 19960221
LR  - 20200304
IS  - 0945-6317 (Print)
IS  - 0945-6317 (Linking)
VI  - 427
IP  - 4
DP  - 1995
TI  - Ultrastructure and immunoreactivity of dystrophic axons indicate a different 
      pathogenesis of Hallervorden-Spatz disease and infantile neuroaxonal dystrophy.
PG  - 415-21
AB  - An immunohistochemical and ultrastructural analysis of dystrophic axons (DAs) in 
      the brain and peripheral nerve of a patient with familial infantile neuroaxonal 
      dystrophy (INAD) and in the brain of a patient with familial Hallervorden-Spatz 
      Disease (HSD) revealed prevalent membrano-tubular or granulo-vesicular profiles 
      with a graded pattern of evolution in INAD, while dense bodies, vesicles and 
      amorphous material were present in HSD. DAs immunoreactivity with tai-protein and 
      200 kDa-neurofilament antibodies was stronger in HSD than in INAD. In both cases 
      immunohistochemistry was positive for ubiquitin and negative for beta-tubulin and 
      beta-amyloid. Distinct ultrastructural features and immunoreactivity pattern of 
      cytoskeletal components suggest different pathogenetic mechanisms.
FAU - Malandrini, A
AU  - Malandrini A
AD  - Institute of Neurological Sciences, University of Siena, Italy.
FAU - Cavallaro, T
AU  - Cavallaro T
FAU - Fabrizi, G M
AU  - Fabrizi GM
FAU - Berti, G
AU  - Berti G
FAU - Salvestroni, R
AU  - Salvestroni R
FAU - Salvadori, C
AU  - Salvadori C
FAU - Guazzi, G C
AU  - Guazzi GC
LA  - eng
PT  - Case Reports
PT  - Comparative Study
PT  - Journal Article
PL  - Germany
TA  - Virchows Arch
JT  - Virchows Archiv : an international journal of pathology
JID - 9423843
RN  - 0 (Cytoskeletal Proteins)
SB  - IM
MH  - Axons/*chemistry/*ultrastructure
MH  - Brain/pathology
MH  - Child
MH  - Cytoskeletal Proteins/analysis
MH  - Female
MH  - Humans
MH  - Microscopy, Electron
MH  - Muscular Dystrophies/*pathology
MH  - Pantothenate Kinase-Associated Neurodegeneration/*pathology
MH  - Sural Nerve/pathology
EDAT- 1995/01/01 00:00
MHDA- 1995/01/01 00:01
CRDT- 1995/01/01 00:00
PHST- 1995/01/01 00:00 [pubmed]
PHST- 1995/01/01 00:01 [medline]
PHST- 1995/01/01 00:00 [entrez]
AID - 10.1007/BF00199391 [doi]
PST - ppublish
SO  - Virchows Arch. 1995;427(4):415-21. doi: 10.1007/BF00199391.