| Rank |
Title |
Journal |
Year |
PubWeight™‹?› |
|
1
|
The human syndrome of dendritic cell, monocyte, B and NK lymphoid deficiency.
|
J Exp Med
|
2011
|
2.96
|
|
2
|
Role of truncating mutations in MME gene in fetomaternal alloimmunisation and antenatal glomerulopathies.
|
Lancet
|
2004
|
2.82
|
|
3
|
An antibody-deficiency syndrome due to mutations in the CD19 gene.
|
N Engl J Med
|
2006
|
2.77
|
|
4
|
Human memory B cells originate from three distinct germinal center-dependent and -independent maturation pathways.
|
Blood
|
2011
|
2.15
|
|
5
|
Replication history of B lymphocytes reveals homeostatic proliferation and extensive antigen-induced B cell expansion.
|
J Exp Med
|
2007
|
2.05
|
|
6
|
Circulating CD21low B cells in common variable immunodeficiency resemble tissue homing, innate-like B cells.
|
Proc Natl Acad Sci U S A
|
2009
|
1.91
|
|
7
|
CD81 gene defect in humans disrupts CD19 complex formation and leads to antibody deficiency.
|
J Clin Invest
|
2010
|
1.79
|
|
8
|
Human ICOS deficiency abrogates the germinal center reaction and provides a monogenic model for common variable immunodeficiency.
|
Blood
|
2005
|
1.63
|
|
9
|
Ig gene rearrangement steps are initiated in early human precursor B cell subsets and correlate with specific transcription factor expression.
|
J Immunol
|
2005
|
1.61
|
|
10
|
Idiopathic CD4+ T lymphopenia without autoimmunity or granulomatous disease in the slipstream of RAG mutations.
|
Blood
|
2011
|
1.53
|
|
11
|
Anti-TNF treatment blocks the induction of T cell-dependent humoral responses.
|
Ann Rheum Dis
|
2012
|
1.51
|
|
12
|
Comparative analysis of Ig and TCR gene rearrangements at diagnosis and at relapse of childhood precursor-B-ALL provides improved strategies for selection of stable PCR targets for monitoring of minimal residual disease.
|
Blood
|
2002
|
1.36
|
|
13
|
Mutations in ZBTB24 are associated with immunodeficiency, centromeric instability, and facial anomalies syndrome type 2.
|
Am J Hum Genet
|
2011
|
1.24
|
|
14
|
Radiosensitive SCID patients with Artemis gene mutations show a complete B-cell differentiation arrest at the pre-B-cell receptor checkpoint in bone marrow.
|
Blood
|
2002
|
1.23
|
|
15
|
B-cell replication history and somatic hypermutation status identify distinct pathophysiologic backgrounds in common variable immunodeficiency.
|
Blood
|
2011
|
1.19
|
|
16
|
Gross deletions involving IGHM, BTK, or Artemis: a model for genomic lesions mediated by transposable elements.
|
Am J Hum Genet
|
2008
|
1.12
|
|
17
|
Immunoglobulin gene rearrangements and the pathogenesis of multiple myeloma.
|
Blood
|
2007
|
1.11
|
|
18
|
Diagnosing mycobacterial lymphadenitis in children using fine needle aspiration biopsy: cytomorphology, ZN staining and autofluorescence -- making more of less.
|
Diagn Cytopathol
|
2008
|
1.07
|
|
19
|
Homeostatic expansion of autoreactive immunoglobulin-secreting cells in the Rag2 mouse model of Omenn syndrome.
|
J Exp Med
|
2010
|
1.03
|
|
20
|
Heterogeneous clinical presentation in ICF syndrome: correlation with underlying gene defects.
|
Eur J Hum Genet
|
2013
|
1.02
|
|
21
|
Clinical heterogeneity can hamper the diagnosis of patients with ZAP70 deficiency.
|
Eur J Pediatr
|
2008
|
1.00
|
|
22
|
Wiskott-Aldrich syndrome protein-mediated actin dynamics control type-I interferon production in plasmacytoid dendritic cells.
|
J Exp Med
|
2013
|
1.00
|
|
23
|
New insights and unresolved issues regarding insertional mutagenesis in X-linked SCID gene therapy.
|
Mol Ther
|
2007
|
1.00
|
|
24
|
Educational paper: primary antibody deficiencies.
|
Eur J Pediatr
|
2011
|
1.00
|
|
25
|
Genetic and demographic features of X-linked agammaglobulinemia in Eastern and Central Europe: a cohort study.
|
Mol Immunol
|
2009
|
0.97
|
|
26
|
Role of Artemis in DSB repair and guarding chromosomal stability following exposure to ionizing radiation at different stages of cell cycle.
|
Mutat Res
|
2006
|
0.95
|
|
27
|
Involvement of Artemis in nonhomologous end-joining during immunoglobulin class switch recombination.
|
J Exp Med
|
2008
|
0.92
|
|
28
|
PID comes full circle: applications of V(D)J recombination excision circles in research, diagnostics and newborn screening of primary immunodeficiency disorders.
|
Front Immunol
|
2011
|
0.89
|
|
29
|
A reversion of an IL2RG mutation in combined immunodeficiency providing competitive advantage to the majority of CD8+ T cells.
|
Haematologica
|
2013
|
0.88
|
|
30
|
Common variable immunodeficiency and idiopathic primary hypogammaglobulinemia: two different conditions within the same disease spectrum.
|
Haematologica
|
2013
|
0.88
|
|
31
|
Antibody deficiency in patients with ataxia telangiectasia is caused by disturbed B- and T-cell homeostasis and reduced immune repertoire diversity.
|
J Allergy Clin Immunol
|
2013
|
0.88
|
|
32
|
Clinical spectrum of LIG4 deficiency is broadened with severe dysmaturity, primordial dwarfism, and neurological abnormalities.
|
Hum Mutat
|
2013
|
0.87
|
|
33
|
GvHD-associated cytokine polymorphisms do not associate with Omenn syndrome rather than T-B- SCID in patients with defects in RAG genes.
|
Clin Immunol
|
2007
|
0.86
|
|
34
|
TREC Based Newborn Screening for Severe Combined Immunodeficiency Disease: A Systematic Review.
|
J Clin Immunol
|
2015
|
0.84
|
|
35
|
Two SCID cases with Cernunnos-XLF deficiency successfully treated by hematopoietic stem cell transplantation.
|
Pediatr Transplant
|
2011
|
0.83
|
|
36
|
Genetic characteristics of eighty-seven patients with the Wiskott-Aldrich syndrome.
|
Mol Immunol
|
2010
|
0.83
|
|
37
|
Homeostatic and maturation-associated proliferation in the peripheral B-cell compartment.
|
Cell Cycle
|
2007
|
0.80
|
|
38
|
Antibody deficiency due to a missense mutation in CD19 demonstrates the importance of the conserved tryptophan 41 in immunoglobulin superfamily domain formation.
|
Hum Mol Genet
|
2011
|
0.79
|
|
39
|
A novel mutation in CD132 causes X-CID with defective T-cell activation and impaired humoral reactivity.
|
J Allergy Clin Immunol
|
2011
|
0.79
|
|
40
|
No significant prognostic value of normal precursor B-cell regeneration in paediatric acute myeloid leukaemia after induction treatment.
|
Br J Haematol
|
2013
|
0.79
|
|
41
|
Decreased somatic hypermutation induces an impaired peripheral B cell tolerance checkpoint.
|
J Clin Invest
|
2016
|
0.78
|
|
42
|
The defect in humoral immunity in patients with Nijmegen breakage syndrome is explained by defects in peripheral B lymphocyte maturation.
|
Cytometry A
|
2012
|
0.78
|
|
43
|
Checkpoints of B cell differentiation: visualizing Ig-centric processes.
|
Ann N Y Acad Sci
|
2011
|
0.78
|
|
44
|
[CD81 has a key role in B lymphocyte function].
|
Med Sci (Paris)
|
2011
|
0.77
|
|
45
|
Late-onset adenosine deaminase deficiency presenting with Heck's disease.
|
Eur J Pediatr
|
2009
|
0.76
|
|
46
|
Overview of 15-year severe combined immunodeficiency in the Netherlands: towards newborn blood spot screening.
|
Eur J Pediatr
|
2015
|
0.76
|
|
47
|
Persistent subclinical immune defects in HIV-1-infected children treated with antiretroviral therapy.
|
AIDS
|
2015
|
0.76
|
|
48
|
Iris atrophy in a patient with X-linked agammaglobulinemia.
|
Can J Ophthalmol
|
2007
|
0.75
|
|
49
|
Concomitant EBV-related B-cell proliferation and juvenile myelomonocytic leukemia in a 2-year-old child.
|
Leuk Res
|
2007
|
0.75
|
|
50
|
Decrease of skin infiltrating and circulating CCR10+ T cells coincides with clinical improvement after topical tacrolimus in Omenn syndrome.
|
J Invest Dermatol
|
2010
|
0.75
|
|
51
|
Mutations in Bruton's tyrosine kinase impair IgA responses.
|
Int J Hematol
|
2015
|
0.75
|
|
52
|
A novel radiosensitive SCID patient with a pronounced G(2)/M sensitivity.
|
DNA Repair (Amst)
|
2010
|
0.75
|
|
53
|
Real-time quantitative (RQ-)PCR approach to quantify the contribution of proliferation to B lymphocyte homeostasis.
|
Methods Mol Biol
|
2013
|
0.75
|