PMID- 10571017
OWN - NLM
STAT- MEDLINE
DCOM- 19991208
LR  - 20190610
IS  - 0006-3002 (Print)
IS  - 0006-3002 (Linking)
VI  - 1455
IP  - 2-3
DP  - 1999 Oct 8
TI  - Tn-syndrome.
PG  - 255-68
AB  - The idiopathic Tn-syndrome, formerly called 'permanent mixed-field
      polyagglutinability', is a rare hematological disorder characterized by the
      expression of the Tn-antigen on all blood cell lineages. The immunodominant
      epitope of the Tn-antigen is terminal alpha-N-acetylgalactosamine,
      O-glycosidically linked to protein. Normally this residue is 3'-substituted by
      5-galactose thereby forming the core 1 structure known as the
      Thomsen-Friedenreich (TF) antigen (Galbeta1 ==> 3GalNAcalpha1 ==> Thr/Ser). The
      cause of the exposure of the Tn-antigen appears to be due to the silencing of the
      gene expression of beta1,3galactosyltransferase, since treatment of deficient
      Tn(+) lymphocyte T clones with 5'azacytidine or Na butyrate leads to reexpression
      of enzyme activity and the sialylated TF-antigen. The Tn-syndrome is acquired and
      permanent and affects both sexes at any age. Its origin is unknown. Pluripotent
      stem cells are affected since all lineages are involved but each one to a
      variable extent. Therefore, normal cells co-exist with Tn-transformed cells.
      Clinically, patients suffering from the Tn-syndrome appear healthy. Laboratory
      findings usually reveal moderate thrombocyto- and leukopenia and some signs of
      hemolytic anemia not warranting any treatment.
FAU - Berger, E G
AU  - Berger EG
AD  - Institute of Physiology, University of Zurich, Switzerland.
      egberger@physiol.unizh.ch
LA  - eng
PT  - Journal Article
PT  - Review
PL  - Netherlands
TA  - Biochim Biophys Acta
JT  - Biochimica et biophysica acta
JID - 0217513
RN  - 0 (Antibodies, Monoclonal)
RN  - 0 (Antigens, Neoplasm)
RN  - 0 (Antigens, Tumor-Associated, Carbohydrate)
RN  - 0 (Lectins)
RN  - 0 (Tn antigen)
RN  - 0 (sialosyl-Tn antigen)
RN  - 3554-90-3 (Thomsen-Friedenreich antigen)
RN  - EC 2.4.1.- (Galactosyltransferases)
RN  - EC 2.4.1.122 (glycoprotein-N-acetylgalactosamine 3-beta-galactosyltransferase)
RN  - M801H13NRU (Azacitidine)
SB  - IM
MH  - Anemia, Hemolytic/immunology
MH  - Antibodies, Monoclonal
MH  - Antigens, Neoplasm/chemistry/immunology
MH  - Antigens, Tumor-Associated, Carbohydrate/chemistry/*immunology
MH  - Azacitidine
MH  - Blood Cells/*immunology
MH  - Erythrocyte Membrane/immunology
MH  - Galactosyltransferases/deficiency
MH  - Hematologic Diseases/enzymology/*immunology/pathology
MH  - Humans
MH  - Lectins
MH  - Leukopenia/immunology
MH  - Molecular Structure
MH  - Syndrome
MH  - Thrombocytopenia/immunology
RF  - 58
EDAT- 1999/11/26 00:00
MHDA- 1999/11/26 00:01
CRDT- 1999/11/26 00:00
PHST- 1999/11/26 00:00 [pubmed]
PHST- 1999/11/26 00:01 [medline]
PHST- 1999/11/26 00:00 [entrez]
AID - S0925-4439(99)00069-1 [pii]
AID - 10.1016/s0925-4439(99)00069-1 [doi]
PST - ppublish
SO  - Biochim Biophys Acta. 1999 Oct 8;1455(2-3):255-68. doi:
      10.1016/s0925-4439(99)00069-1.