t(7;17)(q32-34;q23) MSI2/?

2005-08-01   Jean-Loup Huret 

1.Genetics, Dept Medical Information, University of Poitiers; CHU Poitiers Hospital, F-86021 Poitiers, France

Clinics and Pathology

Disease

Chronic myelogenous leukemia (CML) in accelerated phase (AP-CML)

Epidemiology

only one case to date, a female patient, aged a 40 at the time of the AP-CML

Prognosis

Chronic phase of CML lasted 2 yrs before progression into an accelerated phase accompanied with the appearance of the t(7;17); 2 mths later, a blast crisis occurred, and the patient died 9 mths after the AP-CML, 33 mths after initial diagnosis of CML.

Cytogenetics

Cytogenetics morphological

at(9;22)(q34;q11) was, indeed, present

Cytogenetics molecular

Cryptic translocation; recognized by multicolor FISH plus locus-specific FISH

Genes Involved and Proteins

Note
The gene involved on chromosome 7 is undefined, due to a lack of material for further analysis, but it seems to be a gene of the plexin family, with, perhaps, a sema domain, a plexin repeat, and an immunoglobulin-like plexin transcription factor domain
Gene name
MSI2 (musashi homolog 2 (drosophila))
Location
17q22
Dna rna description
at least 15 exons; various splicing.
Protein description
possesses 2 RNA recognition motifs; likely to be a RNA binding protein; may play a role in RNA metabolism in the cytoplasm; The mouse homolog is ubiquitous, and particularly active in the brain development.

Bibliography

Pubmed IDLast YearTitleAuthors
156805822005Vertebrate 2xRBD hnRNP proteins: a comparative analysis of genome, mRNA and protein sequences.Akindahunsi AA et al
126491772003A novel gene, MSI2, encoding a putative RNA-binding protein is recurrently rearranged at disease progression of chronic myeloid leukemia and forms a fusion gene with HOXA9 as a result of the cryptic t(7;17)(p15;q23).Barbouti A et al
124071782002RNA-binding protein Musashi family: roles for CNS stem cells and a subpopulation of ependymal cells revealed by targeted disruption and antisense ablation.Sakakibara S et al

Citation

Jean-Loup Huret

t(7;17)(q32-34;q23) MSI2/?

Atlas Genet Cytogenet Oncol Haematol. 2005-08-01

Online version: http://atlasgeneticsoncology.org/haematological/1402/t(7;17)(q32-34;q23)

External Links