Abstract
Proline/arginine-rich end leucine-rich repeat protein (PRELP) belongs to the small leucine-rich proteoglycan (SLRP) family, normally expressed in extracellular matrix of collagen-rich tissues. We have previously reported on another SLRP, fibromodulin (FMOD) in patients with chronic lymphocytic leukemia (CLL). PRELP is structurally similar to FMOD with adjacent localization on chromosome 1 (1q32.1). As cluster-upregulation of genes may occur in malignancies, the aim of our study was to analyze PRELP expression in CLL. PRELP was expressed (RT-PCR) in all CLL patients (30/30), as well as in some patients with mantle cell lymphoma (3/5), but not in healthy donor leukocytes (0/20) or tumor samples from other hematological malignancies (0/35). PRELP was also detected in CLL cell-lines (4/4) but not in cell-lines from other hematological tumors (0/9). PRELP protein was detected in all CLL samples but not in normal leukocytes. Deglycosylation experiments revealed a CLL-unique 38 kDa core protein, with an intact signal peptide. This 38 kDa protein was, in contrast to the normal 55 kDa size, not detected in serum which, in combination with the uncleaved signal peptide, suggests cellular retention. The unique expression of a 38 kDa PRELP in CLL cells may suggest involvement in the pathobiology of CLL and merits further studies.
Publication types
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Research Support, Non-U.S. Gov't
MeSH terms
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Adult
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Aged
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Aged, 80 and over
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Antibody Specificity / immunology
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Blotting, Western
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Case-Control Studies
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Cell Line, Tumor
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Extracellular Matrix Proteins / blood
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Extracellular Matrix Proteins / genetics*
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Extracellular Matrix Proteins / immunology
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Female
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Gene Expression Regulation, Leukemic
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Glycoproteins / blood
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Glycoproteins / genetics*
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Glycoproteins / immunology
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Glycosylation
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Humans
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Leukemia, Lymphocytic, Chronic, B-Cell / blood
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Leukemia, Lymphocytic, Chronic, B-Cell / genetics*
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Leukemia, Lymphocytic, Chronic, B-Cell / immunology
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Leukemia, Lymphocytic, Chronic, B-Cell / pathology*
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Leukocytes, Mononuclear / metabolism
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Male
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Middle Aged
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Protein Isoforms / blood
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Protein Isoforms / genetics
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Protein Isoforms / immunology
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Protein Isoforms / metabolism
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Recombinant Proteins / metabolism
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Reverse Transcriptase Polymerase Chain Reaction
Substances
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Extracellular Matrix Proteins
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Glycoproteins
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PRELP protein, human
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Protein Isoforms
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Recombinant Proteins
Grants and funding
The study was supported by grants from the Swedish Cancer Society, the Stockholm County Council, the Torsten and Ragnar Söderberg Foundation, the Cancer Society in Stockholm, the King Gustaf V Jubilee Fund, the Cancer and Allergy Foundation, the Swedish Research Council/SIDA/SAREC, Felix Mindus Research Foundation, EU-grant (EUCAAD-200755) the Iranian Ministry of Health and Medical Education, the Cancer and Allergy Foundation, and the Karolinska Institute Foundations. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.