The proteins involved in mitochondrial mRNA processing and degradation in higher plants have yet to be identified. As a first step towards this aim, we report here the characterisation of a nuclear-encoded DExH box RNA helicase (AtSUV3) localised in Arabidopsis thaliana mitochondria. The AtSUV3 mRNA is assembled from the 16 exons of a weakly expressed unique gene and the predicted protein has a calculated molecular weight of 63.6 kDa. Subcellular fractionation of transgenic plants expressing AtSUV3/GUS fusion proteins localises this protein in mitochondria. The N-terminal domain of AtSUV3 containing the motifs characteristic of DExH box RNA helicases exhibits a low endogenous ATPase activity in vitro which can be stimulated by the presence of mitochondrial RNA, confirming that AtSUV3 is an RNA helicase.z 1999 Federation of European Biochemical Societies.
We used the polymerase chain reaction (PCR) to detect ALL1-AF4 rearrangements, the molecular hallmark of t(4;11) in a series of 46 pre- pre-B (CD19+, CD24+, CD10/CD20/cylgM/sIgM-) acute lymphoblastic leukemias (ALL). Eighteen patients (39%) exhibited fusion transcripts including 4 of 12 children and 14 of 34 adults. This genetic defect was associated with hyperleukocytosis (median leukocyte count 176 x 10(9)/L) and expression of myeloid-associated antigens (CDw65+). In contrast, only two patients from a group of 67 common (CD19/CD10+, cylgM/sIgM-) and pre-B ALLs (CD19/cylgM+, CD10 +/-, sIgM-) showed ALL1- AF4 mRNA. All PCR-positive cases showed multiple amplification products representing alternative splicing events. Moreover, reciprocal der (4)- derived AF4-ALL1 transcripts were observed in 65% of the cases analyzed. Eight of the 18 pre-pre-B ALL patients with an ALL1-AF4 recombination are currently in complete continuous remission for up to 54 months (median, 26 months). Twelve remission samples were available from seven cases, and all of them lacked evidence of minimal residual disease. Overall this study documents a similarly high incidence of ALL1-AF4 recombinations in children (infants excluded) and adults with pre-pre-B ALL and demonstrates the decline of the leukemic cell clone below the detection level of PCR in a remarkable proportion of patients under intense treatment protocols.
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