A synthetic peptide aa region 300-340 of rat Protein Quaking (QKI) conjugated to an immunogenic carrier protein was used as the antigen.
FUNCTION: TRNA-binding protein that plays a central role in myelinization. Binds to the 5'-NACUAAY-N(1,20)-UAAY-3' RNA core sequence. Acts by regulating pre-mRNA splicing, mRNA export, mRNA stability and protein translation. Required to protect and promote stability of mRNAs such as MBP and CDKN1B. Regulator of oligodendrocyte differentiation and maturation in the brain that may play a role in myelin and oligodendrocyte dysfunction in schizophrenia. Participates in mRNA transport by regulating the nuclear export of MBP mRNA. Also involved in regulation of mRNA splicing of MAG pre-mRNA. Acts as a translational repressor.
Note=Defects in Qki are the cause of quakingviable (qkv). Qkv is a spontaneous mutation resulting in hypomyelinization of the central and peripheral nervous systems. Mutant mice develop normally until postnatal day 10 when they display rapid tremors or 'quaking' that is especially pronounced in hindlimbs and experience convulsive tonic-clonic seizures as they mature. Mice with qkv specifically lack isoform3 and isoform4 in myelin-forming cells, while isoform1 is lacking in oligodendrocytes of severely affected tracts. Mice with qkv also lack the Park2 gene product, suggesting that the absence of Park2 may also affect the phenotype.
SUBCELLULAR LOCATION: Nucleus, cytoplasm
TISSUE SPECIFICITY: Highly expressed in myelin-forming cells. Expressed in oligodendrocytes and astrocytes in the central nervous system as well as Schwann cells in the peripheral nervous system. Also expressed in the yolk sac endoderm, adjacent to the mesodermal site of developing blood islands, where the differentiation of blood and endothelial cells first occurs (at protein level). Expressed in brain, lung, heart and testis.
Expressed in the frontal cortex of brain. Down-regulated in the brain of schizophrenic patients.
Maintain the lyophilised/reconstituted antibodies frozen at -20°C for long term storage and refrigerated at 2-8°C for a shorter term. When reconstituting, glycerol (1:1) may be added for an additional stability. Avoid freeze and thaw cycles.
12 months after reconstitution
This item will be shipped to you at ambient temperature in a lyophilised form.
1. Ebersole T.A, et al. Nat. Genet. 12:260-265(1996)
5. Larocque D, et al. Neuron 36:815-829(2002)
For research use only
NZ white rabbit
Polyclonal, whole serum
IHC, WB (confirmed by recombinant protein). A dilution of 1: 300 to 1: 2000 is recommended. The optimal dilution should be determined by the end user. Not yet tested in other applications.
Specific for QKI, isoforms 3, 5, 6,7.
Human, rat, mouse. Other species not yet tested.
Reconstitute in 100 µl of sterile water. Centrifuge to remove any insoluble material.