A new familial immunodeficiency disorder characterized by severe neutropenia, a defective marrow release mechanism, and hypogammaglobulinemia.
about
AMD3100 is a potent antagonist at CXCR4(R334X) , a hyperfunctional mutant chemokine receptor and cause of WHIM syndromeDiversity and Inter-Connections in the CXCR4 Chemokine Receptor/Ligand Family: Molecular PerspectivesPrimary immunodeficiency update: Part II. Syndromes associated with mucocutaneous candidiasis and noninfectious cutaneous manifestationsSrc-mediated phosphorylation of mammalian Abp1 (DBNL) regulates podosome rosette formation in transformed fibroblastsLive imaging of neutrophil motility in a zebrafish model of WHIM syndromeFamilial occurrence of warts, hypogammaglobulinemia, infections, and myelokathexis (WHIM) syndromeOligoclonality, impaired class switch and B-cell memory responses in WHIM syndromeThe CXCR4 antagonist plerixafor corrects panleukopenia in patients with WHIM syndrome.A phase 1 clinical trial of long-term, low-dose treatment of WHIM syndrome with the CXCR4 antagonist plerixaforHierarchical organization of multi-site phosphorylation at the CXCR4 C terminus.Chromothriptic cure of WHIM syndrome.Leukemia in severe congenital neutropenia: defective proteolysis suggests new pathways to malignancy and opportunities for therapy.Syndromic immunodeficiencies: genetic syndromes associated with immune abnormalities.A history of pediatric immunology.CXCR4 mutations in WHIM syndrome: a misguided immune system?Neutrophils in host defense: new insights from zebrafish.WHIM syndrome caused by a single amino acid substitution in the carboxy-tail of chemokine receptor CXCR4.Chromothriptic cure of WHIM syndrome: Implications for bone marrow transplantationCoxsackievirus B3 infects the bone marrow and diminishes the restorative capacity of erythroid and lymphoid progenitors.Description and outcome of a cohort of 8 patients with WHIM syndrome from the French Severe Chronic Neutropenia RegistryAn antedrug of the CXCL12 neutraligand blocks experimental allergic asthma without systemic effect in miceWHIM syndrome: congenital immune deficiency disease.Neutropenia in primary immunodeficiencyA quadrivalent HPV vaccine induces humoral and cellular immune responses in WHIM immunodeficiency syndrome.The EVER proteins as a natural barrier against papillomaviruses: a new insight into the pathogenesis of human papillomavirus infections.Neutropenia and primary immunodeficiency diseases.Genetics on a WHIM.MIM regulates the trafficking of bone marrow cells via modulating surface expression of CXCR4.Harnessing CXCR4 antagonists in stem cell mobilization, HIV infection, ischemic diseases, and oncology.Altered chemotactic response to CXCL12 in patients carrying GATA2 mutations.WHIM Syndrome Caused by Waldenström's Macroglobulinemia-Associated Mutation CXCR4 (L329fs).New monogenic disorders identify more pathways to neutropenia: from the clinic to next-generation sequencing.Pathogenesis, diagnosis and therapeutic strategies in WHIM syndrome immunodeficiency.Mechanisms of Sustained Neutrophilia in Patient WHIM-09, Cured of WHIM Syndrome by Chromothripsis.
P2860
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P2860
A new familial immunodeficiency disorder characterized by severe neutropenia, a defective marrow release mechanism, and hypogammaglobulinemia.
description
1990 nî lūn-bûn
@nan
1990 թուականի Նոյեմբերին հրատարակուած գիտական յօդուած
@hyw
1990 թվականի նոյեմբերին հրատարակված գիտական հոդված
@hy
1990年の論文
@ja
1990年論文
@yue
1990年論文
@zh-hant
1990年論文
@zh-hk
1990年論文
@zh-mo
1990年論文
@zh-tw
1990年论文
@wuu
name
A new familial immunodeficienc ...... sm, and hypogammaglobulinemia.
@ast
A new familial immunodeficienc ...... sm, and hypogammaglobulinemia.
@en
A new familial immunodeficienc ...... sm, and hypogammaglobulinemia.
@nl
type
label
A new familial immunodeficienc ...... sm, and hypogammaglobulinemia.
@ast
A new familial immunodeficienc ...... sm, and hypogammaglobulinemia.
@en
A new familial immunodeficienc ...... sm, and hypogammaglobulinemia.
@nl
prefLabel
A new familial immunodeficienc ...... sm, and hypogammaglobulinemia.
@ast
A new familial immunodeficienc ...... sm, and hypogammaglobulinemia.
@en
A new familial immunodeficienc ...... sm, and hypogammaglobulinemia.
@nl
P2093
P1476
A new familial immunodeficienc ...... sm, and hypogammaglobulinemia.
@en
P2093
Gutterman JU
Kleinerman ES
Kurzrock R
P304
P356
10.1016/0002-9343(90)90187-I
P407
P577
1990-11-01T00:00:00Z