XB-ART-8511
Neurochem Res
June 1, 2001;
26
(6):
703-12.
Rapid functional analysis in Xenopus oocytes of Po protein adhesive interactions.
Yoshida M
,
Colma DR
.
Abstract
We have developed a coupled Xenopus
oocyte expression system for evaluating the functional effects of mutations in known or suspected adhesion molecules, which allows for a very rapid assessment of intercellular adhesion. As a model protein, we first used Protein zero (Po), an adhesion molecule that mediates self-adhesion of the Schwann cell
plasma membrane to form compact myelin in the mammalian
PNS. A wide variety of mutations in Po cause certain human peripheral neuropathies, such as the Charcot-Marie-
Tooth disease (CMT) type 1B and Dejerine-Sottas syndrome (
DSS). After wild-type Po mRNA is injected, the protein is synthesized and correctly targeted to the
oocyte cell surface. When two oocytes are paired, wild-type Po redistributes and concentrates at the cell-cell apposition region, and by electron microscopy, the
oocyte pairs show close cell-cell appositions and are devoid of the microvilli that are observed in uninjected
oocyte pairs. These are hallmark features of highly adhesive cell:cell interfaces. Several point mutations in Po were engineered, corresponding to the molecular defects in the CMT type 1B or
DSS. The proteins encoded by these mutations reached the cell surface but failed to concentrate at the
oocyte interface. Po carrying a point mutation that is found in
DSS is not targeted on the
plasma membrane and fail to accumulate at the cell-cell contact site.
PubMed ID:
11519730
Article link:
Neurochem Res
Grant support:
[+]
Species referenced:
Xenopus laevis
Genes referenced:
egr2
mpz
pmp22
prx
GO keywords:
apical junction complex
[+]
Disease Ontology terms:
Charcot-Marie-Tooth disease type 1B
OMIMs:
CHARCOT-MARIE-TOOTH DISEASE, DEMYELINATING, TYPE 1B; CMT1B
[+]
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