PMID- 23458271
OWN - NLM
STAT- MEDLINE
DCOM- 20131115
LR  - 20130829
IS  - 0722-5091 (Print)
IS  - 0722-5091 (Linking)
VI  - 32
IP  - 5
DP  - 2013 Sep-Oct
TI  - Different profiles of onion bulb in CIDP and CMT1A in relation to extracellular 
      matrix.
PG  - 406-12
LID - 10.5414/NP300597 [doi]
AB  - Hypertrophic neuropathy is usually intractable, and chronic inflammatory 
      demyelinating polyneuropathy (CIDP) and Charcot-Marie-Tooth disease Type 1A 
      (CMT1A) are the representative disorders. The two disorders are sometimes 
      confused both clinically and pathologically. The aim of this study was to clarify 
      the differences in the pathology of large onion bulbs, focusing on the 
      extracellular matrix (ECM) proteins. Nine patients with CIDP and 14 with CMT1A 
      were included. The opened interspaces in OB were frequently shown in CMT1A 
      patients. In CIDP, interspaces of OB packed with collagen fibers were prominent. 
      The mean ratio of opened OB was significantly increased in CMT1A (37.9%) compared 
      to CIDP patients (10.6%) (p = 0.003). Among the ECM examined, tenascin-C (TNC) 
      showed a distinct difference in the pattern of immunoreactivity of OB. The mean 
      ratio of OB showing TNC immunoreactivity was significantly larger in CIDP (29.7%, 
      p = 0.005) than in CMT1A (5.0%). TNC immunoreactivity was confined to the area 
      around myelin sheaths in CMT1A. The increased deposition of collagen fibers in 
      CIDP suggests the activity of nerve regeneration. TNC expression in Schwann cell 
      lamellae comprising OB may also suggest the activity of regeneration. Schwann 
      cell phenotypes in CIDP may be different from CMT1A regarding the production of 
      ECM proteins.
FAU - Oka, Nobuyuki
AU  - Oka N
FAU - Kawasaki, Teruaki
AU  - Kawasaki T
FAU - Unuma, Tsuneo
AU  - Unuma T
FAU - Shigematsu, Kazuo
AU  - Shigematsu K
FAU - Sugiyama, Hiroshi
AU  - Sugiyama H
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - Germany
TA  - Clin Neuropathol
JT  - Clinical neuropathology
JID - 8214420
RN  - 0 (S100 Proteins)
RN  - 0 (Tenascin)
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Aged
MH  - Charcot-Marie-Tooth Disease/metabolism/*pathology
MH  - Extracellular Matrix/metabolism/*pathology
MH  - Female
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Myelin Sheath/metabolism/pathology
MH  - Nerve Fibers, Myelinated/metabolism/*pathology
MH  - Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/metabolism/*pathology
MH  - S100 Proteins/metabolism
MH  - Schwann Cells/metabolism/*pathology
MH  - Tenascin/metabolism
EDAT- 2013/03/06 06:00
MHDA- 2013/11/16 06:00
CRDT- 2013/03/06 06:00
PHST- 2013/08/23 00:00 [accepted]
PHST- 2013/03/06 06:00 [entrez]
PHST- 2013/03/06 06:00 [pubmed]
PHST- 2013/11/16 06:00 [medline]
AID - 10489 [pii]
AID - 10.5414/NP300597 [doi]
PST - ppublish
SO  - Clin Neuropathol. 2013 Sep-Oct;32(5):406-12. doi: 10.5414/NP300597.