Introduction
an RNA-binding protein belonging to a growing
protein family called STAR (signal transduction and activator of
RNA), and essential for myelination in the central nervous
system (CNS).
PMID: 10506177,
PMID: 12682013
a new candidate gene for schizophrenia
involved in myelination.
PMID: 16342280,
PMID: 16641098
QKI levels may regulate oligodendrocyte
differentiation and maturation in human brain, in a similar way
as in mouse.
PMID: 16641098
involved in glial cell differentiation in
mice.
PMID: 14706070
quaking proteins (QKI-5, QKI-6, and QKI-7)
are essential for embryogenesis and myelination.
PMID: 10535969
play key roles in controlling the homeostasis
of their mRNA targets.
PMID: 17079655
Normal Expression
QKI proteins are expressed by differentiated
glia and have been implicated as regulators of myelination, but
are also thought to function during early neural development.
PMID: 9778149
Using X-gal and immunohistochemical staining,
qkI is first shown to be expressed in endothelial cells and
smooth muscle cells.
PMID: 14706070
We show that in mature oligodendrocytes in
culture, the localization of cytoplasmic QKI isoforms requires
an intact cytoskeleton.
PMID: 11178867
Three QKI isoforms have different carboxyl
termini and different subcellular localization. Here, using GFP
fusion protein, we identified a 7-amino acid novel nuclear
localization sequence in the carboxyl terminus of QKI-5, which
is conserved in a subclass of STAR proteins containing SAM68 and
ETLE/T-STAR.
PMID: 10506177
Although the qkI messages and their products
are abundant in brain and heart, a lower level of expression was
found in various other tissues tested.
PMID: 10384037
QKI proteins are expressed in neural
progenitors of the ventricular zone (vz) during murine CNS
development, but that their expression is down-regulated during
neuronal differentiation. By contrast, neural progenitors
located in specific subdomains of the vz maintain expression of
QKI proteins as they differentiate and migrate away into the
emerging nervous system. These QKI+ cells have characteristics
consistent with the acquisition of a glial rather than neuronal
fate.
PMID: 9778149
Antibodies raised to the unique carboxy
peptides of the resulting QKI proteins reveal that, in the
nervous system, all three QKI proteins are expressed strongly in
myelin-forming cells and also in astrocytes. Interestingly,
individual isoforms show distinct intracellular distributions:
QKI-6 and QKI-7 are localized to perikaryal cytoplasm, whereas
QKI-5 invariably is restricted to the nucleus, consistent with
the predicted role of QKI as an RNA-binding protein. In
quakingviable mutants, which display severe dysmyelination,
QKI-6 and QKI-7 are absent exclusively from myelin-forming
cells. By contrast, QKI-5 is absent only in oligodendrocytes of
severely affected tracts.
PMID: 8987822
Expression Alteration
transition from premyelinating
oligodendrocytes into myelin-bearing cells is accompanied by a
dramatic upregulation in expression of the RNA binding QKI
proteins.
PMID: 11178867
In vitro, neural progenitor cells also
down-regulate QKI expression as they differentiate into neurons,
but not if they differentiate into glia.
PMID: 9778149
Function
QKI protein has a critical regulatory role in
smooth muscle cell development.
PMID: 14706070
using an interspecies heterokaryon assay, we
found that QKI-5, but not another STAR protein ETLE, shuttles
between the nucleus and the cytoplasm, which suggests that QKI-5
functions in both cell compartments.
PMID: 10506177
QKI is absent in neurons, QKI-dependent
stabilization of MAP1B mRNA provides a novel mechanism for
advancing MAP1B expression specifically in oligodendroglia
during brain development.
PMID: 16855020
QKI-6 is the major isoform responsible for
CNS myelination, which preferentially promotes MBP expression in
oligodendrocytes.
PPMID: 17079655
Applications
Immunohistochemistry (IHC)
Using X-gal and mmunohistochemical staining,
qkI is first shown to be expressed in endothelial cells and
smooth muscle cells.
PMID: 14706070
IIn the qk(v)/qk(v) brain, immunostaining of
QKI proteins is diminished in an isoform-differential manner
with undefined mechanisms. We examined the expression of QKI
protein isoforms and qkI mRNA isoforms in the qk(v)/qk(v)
mutants and the non-phenotypic wt/qk(v) littermates.
PMID: 12888522