Deregulation of the carbohydrate (chondroitin 4) sulfotransferase 11 (CHST11) gene in a B-cell chronic lymphocytic leukemia with a t(12;14)(q23;q32) Journal Article


Authors: Schmidt, H. H.; Dyomin, V. G.; Palanisamy, N.; Itoyama, T.; Nanjangud, G.; Pirc-Danoewinata, H.; Haas, O. A.; Chaganti, R. S. K.
Article Title: Deregulation of the carbohydrate (chondroitin 4) sulfotransferase 11 (CHST11) gene in a B-cell chronic lymphocytic leukemia with a t(12;14)(q23;q32)
Abstract: The t(12;14)(q23;q32) breakpoints in a case of B-cell chronic lymphocytic leukemia (B-CLL) were mapped by fluorescence in situ hybridization (FISH) and Southern blot analysis and cloned using an IGH switch-γ probe. The translocation affected a productively rearranged IGH allele and the carbohydrate (chondroitin 4) sulfotransferase 11 (CHST11) locus at 12q23, with a reciprocal break in intron 2 of the CHST11 gene. CHST11 belongs to the HNK1 family of Golgi-associated sulfotransferases, a group of glycosaminoglycan-modifying enzymes, and is expressed mainly in the hematopoietic lineage. Northern Blot analysis of tumor RNA using CHST11-specific probes showed expression of two CHST11 forms of abnormal size. 5′ and 3′-Rapid Amplification of cDNA Ends (RACE) revealed IGH/CHST11 as well as CHST11/IGH fusion RNAs expressed from the der(14) and der(12) chromosomes. Both fusion species contained open reading frames making possible the translation of two truncated forms of CHST11 protein. The biological consequence of t(12;14)(q23;q32) in this case presumably is a disturbance of the cellular distribution of CHST11 leading to deregulation of a chondroitin-sulfate-dependent pathway specific to the hematopoietic lineage.
Keywords: controlled study; middle aged; leukemia; unclassified drug; gene translocation; human cell; genetics; case report; molecular genetics; polymerase chain reaction; chromosome 12; gene; gene expression; cytogenetics; rna; gene expression regulation; fluorescence in situ hybridization; dna; immunoglobulin heavy chain; molecular sequence data; nucleotide sequence; tumor cell; chromosome breakage; chromosome rearrangement; base sequence; translocation, genetic; chronic lymphatic leukemia; gene expression regulation, leukemic; b cell leukemia; golgi complex; translocation; chromosomes, human, pair 12; chondroitin sulfate; chromosomes, human, pair 14; chromosome 14; humans; human; male; priority journal; article; b-cll; carbohydrate (chondroitin 4) sulfotransferase 11; chst11; golgi retention; carbohydrate sulfotransferase 11; chondroitin 4 sulfate; sulfotransferase; chst11 protein, human; chromosome translocation 12; chst11 gene; leukemia, b-cell, chronic; sulfotransferases
Journal Title: Oncogene
Volume: 23
Issue: 41
ISSN: 0950-9232
Publisher: Nature Publishing Group  
Date Published: 2004-09-09
Start Page: 6991
End Page: 6996
Language: English
DOI: 10.1038/sj.onc.1207934
PROVIDER: scopus
PUBMED: 15273723
DOI/URL:
Notes: Oncogene -- Cited By (since 1996):8 -- Export Date: 16 June 2014 -- CODEN: ONCNE -- Molecular Sequence Numbers: GENBANK: AY533201, AY533202, AY533203, AY533204, AY533205, NM_018413; -- Source: Scopus
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  1. Vadim G Dyomin
    16 Dyomin
  2. Raju S K Chaganti
    391 Chaganti