Keywords: نقص ایمنی مرکب شدید; common variable immunodeficiency; immunoglobulins; lymphocyte subsets; primary immunodeficiency; severe combined immunodeficiency;
مقالات ISI نقص ایمنی مرکب شدید (ترجمه نشده)
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Keywords: نقص ایمنی مرکب شدید; γc; common gamma chain, CD132; IL; interleukin; sγc; soluble form of common gamma chain; JAK; Janus-activated kinase; STAT; signal transducer and activator of transcription; T-ALL; T-cell acute lymphocytic leukemia; B-ALL; B-cell acute lymphocytic leuke
Keywords: نقص ایمنی مرکب شدید; JAK; Janus kinase; STAT; signal transducers and activators of transcription; EPOR; erythropoietin receptor; TPOR; thrombopoietin receptor; G-CSFR; granulocyte colony stimulating factor receptor; γc; common gamma chain; SCID; severe combined immunodeficie
Keywords: نقص ایمنی مرکب شدید; Immunology; primary immunodeficiency; whole-exome sequencing; hematopoietic stem cell transplantation; common variable immunodeficiency; severe combined immunodeficiency; hyper-IgE syndrome; AIP1; Actin-interacting protein 1; APDS; Activated PI3Kδ syndro
Keywords: نقص ایمنی مرکب شدید; IgE; mast cells; anaphylaxis; humanized mice; tryptase; peanut allergy; APC; Antigen-presenting cell; HSC; Hematopoietic stem cell; huNSG; Humanized nonobese diabetic severe combined immunodeficient common gamma chain-deficient stem cell factor; NOD; No
Keywords: نقص ایمنی مرکب شدید; ADCC; antibody-dependent cell-mediated cytotoxicity; AP3; adaptor protein 3; CHS; Chediak-Higashi syndrome; CMV; cytomegalovirus; CNKD; classical natural killer cell deficiency; CrkL; chicken tumor virus number 10 regulator of kinase-like; CTL; cytotoxi
Keywords: نقص ایمنی مرکب شدید; Primary immunodeficiency disease; whole-exome sequencing; copy number variants; AD; Autosomal dominant; ALPS; Autoimmune lymphoproliferative syndrome; AR; Autosomal recessive; BCM; Baylor College of Medicine; BHCMG; Baylor-Hopkins Center for Mendelian Gen
Keywords: نقص ایمنی مرکب شدید; Gene therapy; Severe combined immunodeficiency; Retroviruses; Adeno-associated viruses; Chimeric antigen receptors (CAR); B-cell leukemia; Gene editing; Thérapie génique; Immunodéficience combinée sévère; Rétrovirus; Virus adéno-déficient; Récep
Keywords: نقص ایمنی مرکب شدید; APC; allophycocyanin; DC; dendritic cells; GARP; Glycoprotein A Repetitions Predominant; IMDM; Iscove modified Dulbecco medium; NOD; nonobese diabetic; PAF; platelet-activating factor; PE; phycoerythrin; SCID; severe combined immunodeficiency; SIT; allerg
Keywords: نقص ایمنی مرکب شدید; Primary immunodeficiency disorder; hematopoietic stem cell transplantation; chimerism; lineage specific; reduced intensity; aGVHD; Acute graft-versus-host disease; BM; Bone marrow; CC; Complete chimerism; cGVHD; Chronic graft-versus-host disease; DLI; Don
Keywords: نقص ایمنی مرکب شدید; Neonatal immune system; Primary immunodeficiency; Severe combined immunodeficiency; T-cell receptor excision circle; Supportive care
Keywords: نقص ایمنی مرکب شدید; Immunology; primary immunodeficiency; whole-exome sequencing; hematopoietic stem cell transplantation; common variable immunodeficiency; severe combined immunodeficiency; hyper-IgE syndrome; AD; Autosomal dominant; AR; Autosomal recessive; BCL10; B-cell l
Keywords: نقص ایمنی مرکب شدید; Primary immunodeficiency; Immune dysregulation; Vaccination; Inactivated vaccines; Live vaccines; HIB; Hemophilus influenza type b; MMR; measles/mumps/rubella; SCID; severe combined immunodeficiency;
Keywords: نقص ایمنی مرکب شدید; WHO; World Health Organization; TNF; tumor necrosis factor; IL; interleukin; HSC; hematopoietic stem cell; NSG; NOD/SCID/γcâ/â; NK; natural killer; DC; dendritic cell; TCR; T cell receptor; DTH; delayed type hypersensitivity; HLA; human leukocyte ant
Keywords: نقص ایمنی مرکب شدید; Severe combined immunodeficiency; severe combined immunodeficiency newborn screening; next-generation sequencing; molecular diagnostics; AT; Ataxia telangiectasia; CGH; Comparative genomic hybridization; CHARGE; Coloboma, heart defects, choanal atresia, g
Keywords: نقص ایمنی مرکب شدید; Autoimmunity; primary immunodeficiency diseases; BÂ cells; T cells; AID; Activation-induced cytidine deaminase; AIHA; Autoimmune hemolytic anemia; AIRE; Autoimmune regulator; ALPS; Autoimmune lymphoproliferative disease; ANA; Anti-nuclear autoantibody; AP
Keywords: نقص ایمنی مرکب شدید; SCID; severe combined immunodeficiency disorders; OS; Omenn syndrome; RAG; Recombination Activating Gene; BMT; bone marrow transplant; CVS; chorionic villous sampling; Severe combined immunodeficiency; Omenn syndrome; RAG; Prenatal diagnosis; Egypt;
Keywords: نقص ایمنی مرکب شدید; Severe combined immunodeficiency; radiation sensitivity; hematopoietic cell transplantation; nonhomologous end joining; DNA repair; ART-SCID; Artemis-deficient severe combined immunodeficiency; ATG; Antithymocyte globulin; DNA-PKcs; DNA-dependent protein
Keywords: نقص ایمنی مرکب شدید; Immunology; primary immunodeficiency; dedicator of cytokinesis 8; HIV1; recombination-activating gene 1; intravenous immunoglobulin; common variable immunodeficiency; severe combined immunodeficiency; newborn screening; ADA; Adenosine deaminase; ApoE; Apo
Keywords: نقص ایمنی مرکب شدید; DiGeorge syndrome; hematopoietic cell transplantation; primary immunodeficiency; severe combined immunodeficiency; T-cell lymphopenia; T-cell receptor excision circle (TREC);
Keywords: نقص ایمنی مرکب شدید; AAAAI; American Academy of Allergy, Asthma & Immunology; ACAAI; American College of Allergy, Asthma & Immunology; HSCT; Hematopoietic stem cell therapy; JCAAI; Joint Council of Allergy, Asthma & Immunology; PIDD; Primary immunodeficiency disease; SCID; Se
Keywords: نقص ایمنی مرکب شدید; Hereditary folate malabsorption; Severe combined immunodeficiency; Cytokine profile; Pneumocystis pneumonia; Novel mutations;
Keywords: نقص ایمنی مرکب شدید; Severe combined immunodeficiency; conditioning; natural killer cells; chimerism; engraftment; adenosine deaminase deficiency; ADA; Adenosine deaminase deficiency; allo-SCT; Allogeneic hematopoietic stem cell transplantation; JAK3; Janus kinase 3; MFD; Mat
Keywords: نقص ایمنی مرکب شدید; CD; cluster of differentiation; DMEM; dulbecco's modified eagle's medium; FBS; fetal bovine serum; MHC; myosin heavy chain; PBS; phosphate-buffered saline; SCID; severe combined immunodeficiency; SDS; sodium dodecyl sulfate; SMC; smooth muscle cell; S
Keywords: نقص ایمنی مرکب شدید; Purine nucleoside phosphorylase; severe combined immunodeficiency; newborn screening; tandem mass spectrometry; late-onset; delayed-onset; purine nucleoside phosphorylase-combined immunodeficiency; T-cell receptor excision circle; inherited disorder; AC
Keywords: نقص ایمنی مرکب شدید; Severe combined immunodeficiency; hematopoietic cell transplantation; sibling donors; unrelated donors; umbilical cord blood; conditioning; serotherapy; ADA; Adenosine deaminase; aGVHD; Acute graft-versus-host disease; BM; Bone marrow; cGVHD; Chronic graf
Keywords: نقص ایمنی مرکب شدید; Hepatocyte; Liver; Chimera; Humanization; Animal Model; AFC8; FKBP-Caspase 8 gene driven by the albumin promoter; CYP; cytochrome; Fah; fumarylacetoacetate hydrolase; FRG; Fah/Rag2/interleukin common gamma chain knockout; NOD; non-obese diabetic; NTBC; 2-
Newborn screening for severe combined immunodeficiency using a novel and simplified method to measure T-cell excision circles (TREC)
Keywords: نقص ایمنی مرکب شدید; SCID; Severe Combined Immunodeficiency; DBSs; dried blood spots; NBS; newborn screening; TRECs; T-cell receptor excision circles; ADA-SCID; Adenosine deaminase deficiency; JAK3; Janus kinase 3, Tyrosine-protein kinase; ATG; antithymocyte globulin; Newborn
Idiopathic T cell lymphopenia identified in New York State Newborn Screening
Keywords: نقص ایمنی مرکب شدید; TCL; T cell lymphopenia; SCID; severe combined immunodeficiency; TREC; T-cell receptor excision circle; Newborn screening; TREC level; Idiopathic T-cell lymphopenia; Severe combined immunodeficiency;
Humanized chimeric mouse models of hepatitis B virus infection
Keywords: نقص ایمنی مرکب شدید; HBV; hepatitis B virus; cccDNA; covalently closed circular DNA; SCID; severe combined immunodeficiency; alb-uPA; albumin-promoted urokinase-type plasminogen activator; RAG2; recombinant activation gene 2; RI; repopulation index; FAH; fumarylacetoacetate h
MST1 deficiency promotes B cell responses by CD4+ T cell-derived IL-4, resulting in hypergammaglobulinemia
Keywords: نقص ایمنی مرکب شدید; MST1; CD4+ T cell; B cell activation; IL-4; CD40L; Mammalian sterile-20 like kinase 1; (MST1); Severe combined immunodeficiency; (SCID); Regulatory T; (Treg); T follicular helper; (Tfh); Class-switch recombination; (CSR); T cell receptor; (TCR);
Current Knowledge and Priorities for Future Research in Late Effects after Hematopoietic Stem Cell Transplantation (HCT) for Severe Combined Immunodeficiency Patients: A Consensus Statement from the Second Pediatric Blood and Marrow Transplant Consortium
Keywords: نقص ایمنی مرکب شدید; Late effects; Pediatric allogeneic bone marrow transplantation; Severe combined immunodeficiency;
Mutations in linker for activation of TÂ cells (LAT) lead to a novel form of severe combined immunodeficiency
Keywords: نقص ایمنی مرکب شدید; Severe combined immunodeficiency; linker for activation of TÂ cells; immunodeficiency; T-cell receptor signaling; genetic defect; T lymphopenia; GFP; Green fluorescent protein; LAT; Linker for activation of TÂ cells; NK; Natural killer; OMIM; Online Mende
FOXN1 Italian founder mutation in Indian family: Implications in prenatal diagnosis
Keywords: نقص ایمنی مرکب شدید; FOXN1; Forkhead box N1; SCID; severe combined immunodeficiency; DGS; Di-George Syndrome; VUS; variants of unknown significance; FOXN1; Alopecia; Nail dystrophy; T-cell immunodeficiency;
Functionalized 2D nanomaterials for gene delivery applications
Keywords: نقص ایمنی مرکب شدید; 2D; two-dimensional; SCID; Severe Combined Immunodeficiency; CTL; cytotoxic T cell lymphocyte; MHC; major histocompatibility complex; TCR; T cell receptor; JAM1; junctional adhesion molecule 1; HIV-1; human immunodeficiency virus type 1; VSV-G; vesicular
An evaluation of the TREC assay with regard to the integration of SCID screening into the Dutch newborn screening program
Keywords: نقص ایمنی مرکب شدید; Severe combined immunodeficiency; Newborn screening; TREC; T-cell receptor excisions circles; κ-deleting recombination excision circles; Heel prick cards;
CRISPR knock out CTLA-4 enhances the anti-tumor activity of cytotoxic T lymphocytes
Keywords: نقص ایمنی مرکب شدید; CRISPR-Cas9; Clustered regularly interspaced short palindromic repeats and CRISPR-associated protein 9; CTLA-4; cytotoxic T-lymphocyte-associated protein 4; KO; knock out; CTL; cytotoxic T lymphocytes; TNF-α; Tumor necrosis factor-α; IFN-γ; interferon
Development of novel avenues to overcome challenges facing CAR T cells
Keywords: نقص ایمنی مرکب شدید; AICD; activation-induced cell death; AMP; adenosine monophosphate; APC; antigen-presenting cells; ATP; adenosine triphosphate; CAFs; cancer-associated fibroblasts; CAR; chimeric antigen receptor; CEA; carcinoembryonic antigen; CTLA4; cytotoxic T-lymphocyt
Novel RAG1 mutation and the occurrence of mycobacterial and Chromobacterium violaceum infections in a case of leaky SCID
Keywords: نقص ایمنی مرکب شدید; Severe combined immunodeficiency; RAG 1; Leaky SCID;
Research ArticleHuman liver chimeric mice as a new model of chronic hepatitis E virus infection and preclinical drug evaluation
Keywords: نقص ایمنی مرکب شدید; HEV; hepatitis E virus; uPA; urokinase-type plasminogen activator; SCID; severe combined immunodeficiency; PCR; polymerase chain reaction; ORF; open reading frame; UTR; untranslated region, USB, uPA/SCID/beige; i.v.; intravenous; GT; genotype; ELISA; enzy
Adoptive immunotherapy for primary immunodeficiency disorders with virus-specific T lymphocytes
Keywords: نقص ایمنی مرکب شدید; Primary immunodeficiency; immunotherapy; cytotoxic T lymphocytes; antiviral therapy; ADV; Adenovirus; CMV; Cytomegalovirus; EBV-LPD; EBV-related lymphoproliferative disease; GVHD; Graft-versus-host disease; HHV6; Human herpesvirus 6; HSCT; Hematopoietic
Mouse tissue distribution and persistence of the food-born fusariotoxins Enniatin B and Beauvericin
Keywords: نقص ایمنی مرکب شدید; ABC; ATP-binding cassette; Bea; beauvericin; b.w.; bodyweight; DMSO; dimethyl sulfoxide; Enn; Enniatin; ESI; Electrospray ionisation; LC-MS/MS; liquid chromatography-tandem mass spectrometry; p.a.; per analysis; sSRM; scheduled selected reaction monitorin
In vivo anti-tumor activity of the PARP inhibitor niraparib in homologous recombination deficient and proficient ovarian carcinoma
Keywords: نقص ایمنی مرکب شدید; BRCA1/2; BRCA1 or BRCA2; CDK; cyclin-dependent kinase; FBS; heat-inactivated fetal bovine serum; HGSOC; high-grade serous ovarian carcinoma; HR; homologous recombination; IP; intraperitoneal; pADPr; poly(ADP-ribose) polymer; PARP; poly(ADP-ribose) polymer
Original ArticleAppendagesA Guide to Studying Human Hair Follicle Cycling In Vivo
Keywords: نقص ایمنی مرکب شدید; DP; dermal papilla; HF; hair follicles; IRS; inner root sheath; HF-IS; human scalp skin in situ; HF-XG; xenografted anagen HF; SCID; severe combined immunodeficiency;
Recurrent viral infections associated with a homozygous CORO1A mutation that disrupts oligomerization and cytoskeletal association
Keywords: نقص ایمنی مرکب شدید; Coronin-1A; immunodeficiency; T-cell lymphopenia; Arp; Actin-related protein; CC; Coiled-coil leucine zipper; CE; C-terminal extension; CORO1A; Coronin-1A; F-actin; Filamentous actin; FITC; Fluorescein isothiocyanate; MAP; Mitogen-activated protein; NK; N
Agammaglobulinemia associated to nasal polyposis due to a hypomorphic RAG1 mutation in a 12 years old boy
Keywords: نقص ایمنی مرکب شدید; PID; primary immune deficiency; SCID; severe combined immunodeficiency; CVID; common variable immunodeficiency; OS; Omenn syndrome; RAG1; recombination-activating gene 1; RAG2; recombination-activating gene 2; RSSs; recombination signal sequences; DSBs; D
Precision Molecular Diagnosis Defines Specific Therapy in Combined Immunodeficiency with Megaloblastic Anemia Secondary to MTHFD1 Deficiency
Keywords: نقص ایمنی مرکب شدید; Megaloblastic anemia; C-1-Tetrahydrofolate synthase (C1-THF synthase); Folinic acid; Combined immunodeficiency; Lymphopenia; MTHFD1; Methylenetetrahydrofolate dehydrogenase 1; PCP; Pneumocystic jirovecii pneumonia; SCID; Severe combined immunodeficiency;
Immune deficiencies, infection, and systemic immune disordersSystematic neonatal screening for severe combined immunodeficiency and severe T-cell lymphopenia: Analysis of cost-effectiveness based on French real field data
Keywords: نقص ایمنی مرکب شدید; Primary immunodeficiency; severe combined immunodeficiency; severe T-cell lymphopenia; newborn screening; health economics; cost-effectiveness; health policy; CEREDIH; French National Center for Primary Immunodeficiencies; HSCT; Hematopoietic stem cell tr
Brief CommunicationSuccessful newborn screening for SCID in the Navajo Nation
Keywords: نقص ایمنی مرکب شدید; DBS; dried blood spots; dsDNA; double strand DNA; GVHD; graft vs. host disease; HCT; hematopoietic cell transplant; NBS; newborn screening; SCID; severe combined immunodeficiency; SCID-A; Artemis deficient SCID; TCL; T cell lymphopenia; TCR; T cell recept
Positive Family History, Infection, Low Absolute Lymphocyte Count (ALC), and Absent Thymic Shadow: Diagnostic Clues for All Molecular Forms of Severe Combined Immunodeficiency (SCID)
Keywords: نقص ایمنی مرکب شدید; Absent thymic shadow; Failure to thrive infections; Lymphopenia; Severe combined immunodeficiency; ADA; Adenosine deaminase; ALC; Absolute lymphocyte count; JAK3; Janus kinase 3; PJP; Pneumocystis jiroveci pneumonia; RAG; Recombinase-activating genes; SCI