PMID- 22803950
OWN - NLM
STAT- MEDLINE
DCOM- 20130131
LR  - 20120921
IS  - 1399-0039 (Electronic)
IS  - 0001-2815 (Linking)
VI  - 80
IP  - 4
DP  - 2012 Oct
TI  - Killer immunoglobulin-like receptors (KIR) and their HLA-ligands in Italian 
      paroxysmal nocturnal haemoglobinuria (PNH) patients.
PG  - 322-7
LID - 10.1111/j.1399-0039.2012.01932.x [doi]
AB  - Paroxysmal nocturnal haemoglobinuria (PNH) is a haematopoietic disorder 
      characterized by expansion of phosphatidylinositol glycan-A-defective 
      progenitor(s). Immune-dependent mechanisms, likely involving a deranged T 
      cell-dependent autoimmune response, have been consistently associated with the 
      selection/dominance of PNH precursors. Natural killer (NK) lymphocytes might 
      participate in PNH pathogenesis, but their role is still controversial. NK 
      activity is dependent on the balance between activating and inhibiting signals. 
      Key component in such regulatory network is represented by killer 
      immunoglobulin-like receptors (KIR). KIR are also involved in the regulation of 
      adaptive cytotoxic T cell response and associated with autoimmunity. This study 
      investigated on the frequency of KIR genes and their known human leukocyte 
      antigen (HLA) ligands in 53 PNH Italian patients. We observed increased frequency 
      of genotypes characterized by </=2 activating KIR as well as by the presence of an 
      inhibitory/activating gene ratio >/=3.5. In addition, an increased matching between 
      KIR-3DL1 and its ligand HLA-Bw4 was found. These genotypes might be associated 
      with lower NK-dependent recognition of stress-related self molecules; this is 
      conceivable with the hypothesis that an increased availability of specific T cell 
      targets, not cleared by NK cells, could be involved in PNH pathogenesis. These 
      data may provide new insights into autoimmune PNH pathogenesis.
CI  - (c) 2012 John Wiley & Sons A/S.
FAU - Cosentini, E
AU  - Cosentini E
AD  - Dipartimento di Biologia e Patologia Cellulare e Molecolare, Universita di Napoli 
      Federico II, Napoli, Italy.
FAU - Gargiulo, L
AU  - Gargiulo L
FAU - Bruno, P
AU  - Bruno P
FAU - Lastraioli, S
AU  - Lastraioli S
FAU - Risitano, A
AU  - Risitano A
FAU - Camerlingo, R
AU  - Camerlingo R
FAU - Luongo, V
AU  - Luongo V
FAU - Serra, M
AU  - Serra M
FAU - Sica, M
AU  - Sica M
FAU - Garzillo, C
AU  - Garzillo C
FAU - Giani, U
AU  - Giani U
FAU - Notaro, R
AU  - Notaro R
FAU - Alfinito, F
AU  - Alfinito F
FAU - Ruggiero, G
AU  - Ruggiero G
FAU - Terrazzano, G
AU  - Terrazzano G
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20120717
PL  - England
TA  - Tissue Antigens
JT  - Tissue antigens
JID - 0331072
RN  - 0 (HLA-B Antigens)
RN  - 0 (HLA-Bw4 antigen)
RN  - 0 (Ligands)
RN  - 0 (Receptors, KIR3DL1)
SB  - IM
MH  - Adult
MH  - Alleles
MH  - Case-Control Studies
MH  - Female
MH  - Gene Expression
MH  - Gene Frequency
MH  - HLA-B Antigens/*genetics/immunology
MH  - Haplotypes
MH  - Hemoglobinuria, Paroxysmal/*genetics/immunology/pathology
MH  - Humans
MH  - Italy
MH  - Killer Cells, Natural/*immunology/metabolism/pathology
MH  - Ligands
MH  - Male
MH  - Middle Aged
MH  - Molecular Typing
MH  - Receptors, KIR3DL1/*genetics/immunology
MH  - Signal Transduction
MH  - T-Lymphocytes/*immunology/metabolism/pathology
EDAT- 2012/07/19 06:00
MHDA- 2013/02/01 06:00
CRDT- 2012/07/19 06:00
PHST- 2012/04/27 00:00 [received]
PHST- 2012/06/19 00:00 [revised]
PHST- 2012/06/27 00:00 [accepted]
PHST- 2012/07/19 06:00 [entrez]
PHST- 2012/07/19 06:00 [pubmed]
PHST- 2013/02/01 06:00 [medline]
AID - 10.1111/j.1399-0039.2012.01932.x [doi]
PST - ppublish
SO  - Tissue Antigens. 2012 Oct;80(4):322-7. doi: 10.1111/j.1399-0039.2012.01932.x. 
      Epub 2012 Jul 17.