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Hyperphosphorylated paratarg-7: a new molecularly defined risk factor for monoclonal gammopathy of undetermined significance of the IgM type and Waldenström macroglobulinemia
| Content Provider | Scilit |
|---|---|
| Author | Grass, Sandra Preuss, Klaus-Dieter Wikowicz, Alexandra Terpos, Evangelos Ziepert, Marita Nikolaus, Diana Yang, Yin Fadle, Natalie Regitz, Evi Dimopoulos, Meletios Treon, Steven P. Hunter, Zachary R. Pfreundschuh, Michael |
| Copyright Year | 2011 |
| Description | We recently described paratarg-7 (P-7), a protein of unknown function, as the target of 15% of immunoglobulin A (IgA) and IgG paraproteins in monoclonal gammopathy of undetermined significance (MGUS) and multiple myeloma. To determine the frequency of P-7 as a paraprotein target in IgM-MGUS and Waldenström macroglobulinemia (WM), sera from patients with IgM-MGUS/WM were tested for reactivity with recombinant P-7 by enzyme-linked immunoabsorbent assay. The specificity of the paraprotein-mediated reaction was shown by absorption studies and cloning of the respective B-cell receptor. The paraproteins of 18 (9 WM and 9 IgM-MGUS) of 161 patients (11%) reacted with P-7. Isoelectric focusing and phosphatase treatment showed that P-7 was hyperphosphorylated (pP-7) in all patients with an anti–P-7-specific IgM paraprotein tested. Because only 4 of 200 healthy controls (2%) were carriers of pP-7, pP-7 carrier state is associated with a significantly increased risk (odds ratio = 6.2; P = .001) for developing IgM-MGUS/MW. Family analyses showed that the pP-7 carrier state is inherited as a dominant trait. After IgA/IgG-MGUS and multiple myeloma, IgM-MGUS/WM is the second neoplasia associated with pP-7 carrier state. The dominant inheritance of pP-7 explains cases of familial IgM-MGUS/WM and enables the identification of family members at increased risk. |
| Related Links | http://www.bloodjournal.org/content/bloodjournal/117/10/2918.full.pdf |
| Ending Page | 2923 |
| Page Count | 6 |
| Starting Page | 2918 |
| DOI | 10.1182/blood-2010-09-306076 |
| Journal | Blood |
| Issue Number | 10 |
| Volume Number | 117 |
| Language | English |
| Publisher | American Society of Hematology |
| Publisher Date | 2011-03-10 |
| Access Restriction | Open |
| Subject Keyword | Medical Laboratory Technology Multiple Myeloma Inherited Protein Treatment Function Igm Gammopathy Undetermined Paratarg Journal: Blood (Vol- 114, Issue- 10) |
| Content Type | Text |
| Resource Type | Article |
