PRIMARY T-CELL IMMUNODEFICIENCIES

被引:23
作者
FISCHER, A
机构
[1] INSERM U 132, Hôpital des Enfants-Malades, Paris
关键词
D O I
10.1016/0952-7915(93)90040-Y
中图分类号
R392 [医学免疫学]; Q939.91 [免疫学];
学科分类号
100102 ;
摘要
The phenotypes of many primary T-cell immunodeficiencies have been described, including diseases associated with defective T-cell differentiation and/or activation. Recently, genotypes have been defined for some of them, such as X-linked severe combined immunodeficiency and CD3 deficiencies (or hyper IgM syndrome). Phenotype/genotype correlation studies open a fruitful way to a better understanding of primary T-cell immunodeficiencies.
引用
收藏
页码:569 / 578
页数:10
相关论文
共 91 条
[71]   ABNORMAL SIGNAL TRANSDUCTION IN A PATIENT WITH SEVERE COMBINED IMMUNODEFICIENCY DISEASE [J].
RIJKERS, GT ;
SCHARENBERG, JGM ;
VANDONGEN, JJM ;
NEIJENS, HJ ;
ZEGERS, BJM .
PEDIATRIC RESEARCH, 1991, 29 (03) :306-309
[72]   DEPLETION OF CD8+ CELLS IN HUMAN THYMIC MEDULLA RESULTS IN SELECTIVE IMMUNE-DEFICIENCY [J].
ROIFMAN, CM ;
HUMMEL, D ;
MARTINEZVALDEZ, H ;
THORNER, P ;
DOHERTY, PJ ;
PAN, S ;
COHEN, F ;
COHEN, A .
JOURNAL OF EXPERIMENTAL MEDICINE, 1989, 170 (06) :2177-2182
[73]   CD43, A MOLECULE DEFECTIVE IN WISKOTT-ALDRICH SYNDROME, BINDS ICAM-1 [J].
ROSENSTEIN, Y ;
PARK, JK ;
HAHN, WC ;
ROSEN, FS ;
BIERER, BE ;
BURAKOFF, SJ .
NATURE, 1991, 354 (6350) :233-235
[74]   V(D)J RECOMBINATION - BROKEN DNA-MOLECULES WITH COVALENTLY SEALED (HAIRPIN) CODING ENDS IN SCID MOUSE THYMOCYTES [J].
ROTH, DB ;
MENETSKI, JP ;
NAKAJIMA, PB ;
BOSMA, MJ ;
GELLERT, M .
CELL, 1992, 70 (06) :983-991
[75]   THE DNA-BINDING DEFECT OBSERVED IN MAJOR HISTOCOMPATIBILITY COMPLEX CLASS-II REGULATORY MUTANTS CONCERNS ONLY ONE MEMBER OF A FAMILY OF COMPLEXES BINDING TO THE X-BOXES OF CLASS-II PROMOTERS [J].
SANCHEZ, CH ;
REITH, W ;
SILACCI, P ;
MACH, B .
MOLECULAR AND CELLULAR BIOLOGY, 1992, 12 (09) :4076-4083
[76]   T-HELPER TYPE-2-LIKE CELLS AND THERAPEUTIC EFFECTS OF INTERFERON-GAMMA IN COMBINED IMMUNODEFICIENCY WITH HYPEREOSINOPHILIA (OMENNS SYNDROME) [J].
SCHANDENE, L ;
FERSTER, A ;
MASCARTLEMONE, F ;
CRUSIAUX, A ;
GERARD, C ;
MARCHANT, A ;
LYBIN, M ;
VELU, T ;
SARIBAN, E ;
GOLDMAN, M .
EUROPEAN JOURNAL OF IMMUNOLOGY, 1993, 23 (01) :56-60
[77]   SEVERE COMBINED IMMUNODEFICIENCY (SCID) IN MAN - B-CELL NEGATIVE (B-) SCID PATIENTS EXHIBIT AN IRREGULAR RECOMBINATION PATTERN AT THE JH LOCUS [J].
SCHWARZ, K ;
HANSENHAGGE, TE ;
KNOBLOCH, C ;
FRIEDRICH, W ;
KLEIHAUER, E ;
BARTRAM, CR .
JOURNAL OF EXPERIMENTAL MEDICINE, 1991, 174 (05) :1039-1048
[78]  
SIMON HU, 1993, CLIN EXP IMMUNOL, V91, P43
[79]   EVIDENCE FOR DEFECTIVE TRANSMEMBRANE SIGNALING IN B-CELLS FROM PATIENTS WITH WISKOTT-ALDRICH SYNDROME [J].
SIMON, HU ;
MILLS, GB ;
HASHIMOTO, S ;
SIMINOVITCH, KA .
JOURNAL OF CLINICAL INVESTIGATION, 1992, 90 (04) :1396-1405
[80]   INDEPENDENT MUTATIONS OF THE HUMAN CD3-EPSILON GENE RESULTING IN A T-CELL RECEPTOR/CD3 COMPLEX IMMUNODEFICIENCY [J].
SOUDAIS, C ;
DEVILLARTAY, JP ;
LEDEIST, F ;
FISCHER, A ;
LISOWSKAGROSPIERRE, B .
NATURE GENETICS, 1993, 3 (01) :77-81