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Titolo:
ACCUMULATION OF PERIPHERAL MYELIN PROTEIN-22 IN ONION BULBS AND SCHWANN-CELLS OF BIOPSIED NERVES FROM PATIENTS WITH CHARCOT-MARIE-TOOTH DISEASE TYPE 1A
Autore:
NISHIMURA T; YOSHIKAWA H; FUJIMURA H; SAKODA S; YANAGIHARA T;
Indirizzi:
OSAKA UNIV,SCH MED,DEPT NEUROL,YAMADAOKA 2-2 SUITA OSAKA 565 JAPAN OSAKA UNIV,SCH MED,DEPT NEUROL SUITA OSAKA 565 JAPAN
Titolo Testata:
Acta Neuropathologica
fascicolo: 5, volume: 92, anno: 1996,
pagine: 454 - 460
SICI:
0001-6322(1996)92:5<454:AOPMPI>2.0.ZU;2-2
Fonte:
ISI
Lingua:
ENG
Soggetto:
GENE; DUPLICATION; EXPRESSION;
Keywords:
PMP-22; CMT1A; ONION BULB FORMATION; IMMUNOHISTOCHEMISTRY;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Science Citation Index Expanded
Science Citation Index Expanded
Citazioni:
19
Recensione:
Indirizzi per estratti:
Citazione:
T. Nishimura et al., "ACCUMULATION OF PERIPHERAL MYELIN PROTEIN-22 IN ONION BULBS AND SCHWANN-CELLS OF BIOPSIED NERVES FROM PATIENTS WITH CHARCOT-MARIE-TOOTH DISEASE TYPE 1A", Acta Neuropathologica, 92(5), 1996, pp. 454-460

Abstract

Peripheral myelin protein 22 (PMP-22) is a glycoprotein expressed in the myelin sheath of myelinated Schwann cells. Duplication of the PMP-22 gene and its gene dosage effect have been postulated to be involvedin the pathogenesis in the majority of individuals with Charcot-Marie-Tooth disease type IA (CMT1A). Northern blot analysis has demonstrated that the mean relative ratio of PMP-22 mRNA/beta-actin mRNA in biopsied nerves of patients with CMT1A is significantly higher than that indisease controls. To investigate whether the elevated expression of PMP-22 mRNA is reflected in the amount and the localization of PMP-22, we analyzed PMP-22, myelin basic protein (MBP), protein zero (PO), andS-100 immunoreactivities in biopsied nerves from six patients with CMT1A, five patients with other types of CMT, five patients with acquired demyelinating neuropathies, and two normal subjects. In all patientswith CMT other than CMT1A and acquired demyelinating neuropathy, as well as in normal subjects, the myelin sheath was immunoreactive for PMP-22, MBP, and PO, while the Schwann cell cytoplasm was immunoreactiveonly for S-100. In five out of six patients with CMT1A, however, the PMP-22 immunoreactivity was present not only on the myelin sheath but also in the Schwann cell cytoplasm and onion bulbs (OBs). Although OBsare nonspecific and also seen in other inherited or acquired demyelinating neuropathies, the PMP-22-positive OBs were seen exclusively in CMT1A. The finding suggested that the expression of PMP-22 was abnormalfor its localization and probably for the amount in patients with CMT1A carrying duplication of the PMP-22 gene.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 13/08/20 alle ore 14:40:47