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PedAM

Pediatric Disease Annotations & Medicines




Disease severe combined immunodeficiency
Phenotype C0085110|severe combined immunodeficiency
Sentences 101
PubMedID- 24189293 Previous studies demonstrated that cd34− severe combined immunodeficiency (scid)-repopulating cells (srcs) are a distinct class of primitive hscs in comparison to the well-characterized cd34+cd38− srcs.
PubMedID- 22844424 Twenty four five-week old non-obese diabetic/severe combined immunodeficiency (nod/scid) male mice were purchased from the chinese association for laboratory animal science (calas; beijing, china) and housed under specific pathogen-free conditions, in a controlled temperature and humidity environment with 12 h light/dark cycles.
PubMedID- 23343349 Ak 2 mutations cause reticular dysgenesis, the most severe combined immunodeficiency in humans [48].
PubMedID- 26083776 Female non-obese diabetic severe combined immunodeficiency (nod-scid) mice were purchased from clea and maintained in the animal facility of kobe university graduate school of medicine.
PubMedID- 24447304 Eight week old female nonobese diabetic/severe combined immunodeficiency mice (nod/scid; harlan, in) were used as hosts for tumor xenografts.
PubMedID- 24465715 Six-month-old male, severe combined immunodeficiency (scid) mice were housed in a barrier filter room and fed purina rodent chow ad lib.
PubMedID- 23841937 Six- to eight-week-old severe combined immunodeficiency mice were implanted with glioblastoma u87 (n = 8) or adenocarcinoma mda-mb-231 (mda) (n = 11) in the shoulder.
PubMedID- 25944695 Six- to eight-week-old male cb.17/severe combined immunodeficiency disease (scid) mice were inoculated subcutaneously with 5 × 106 oci-ly7 tumor cells in 0.1 ml pbs for tumor development.
PubMedID- 23542179 Four-week male athymic nod cb17-prkdc/scid (severe combined immunodeficiency) mice were purchased from jackson laboratory and maintained in the animal facilities according to the protocol approved by the american association for accreditation of laboratory animal care.
PubMedID- 22829152 The leukemic non-obese diabetic/severe combined immunodeficiency (nod/scid) mouse system with the in vivo combination treatment with imatinib and everolimus showed a decrease of tumor burden including cd34+ cells.
PubMedID- 21750681 The recessive defect ‘severe combined immunodeficiency’ (scid), which is found in the arabian breed, comprises a fatal deficiency in t- and b-lymphocyte numbers and function.
PubMedID- 22363629 We subsequently demonstrated that compared with balb/c mice, mice with severe combined immunodeficiency (scid) were acutely hypersensitive to 34-1-2 s effects indicating that recipient lymphocytes are important in significantly reducing severe lung damage induced by 34-1-2s [21].
PubMedID- 21695116 A variety of mutations are responsible for the scid (severe combined immunodeficiency syndrome) phenotype with a deficiency in different lymphoid cell populations [1].
PubMedID- 22111002 severe combined immunodeficiency (scid) is a rare disease in which the affected organism is unable to mount an immune response due to loss of b and t lymphocytes.
PubMedID- 24497838 severe combined immunodeficiency (scid) beige mice were maintained in a pathogen–free environment under controlled conditions of light and humidity.
PubMedID- 23060872 Immunodeficiencies, such as severe combined immunodeficiency (scid) or intermediate forms of combined immunodeficiency (cid), may present first with gastrointestinal symptoms, often fatal in early childhood if untreated (geha et al., 2007).
PubMedID- 23764770 Female cb17 mice at 6–8 weeks of age with severe combined immunodeficiency (scid) were used for in vivo tumor modeling studies (charles river laboratories inc., holister, ca) and were housed in polycarbonate cages using a hepa-filtered, ventilated rack system (allentown inc., allentown, nj).
PubMedID- 20051957 A total of 20 nod/scid (non-obese diabetic–severe combined immunodeficiency) female mice had 8 × 106 mda-mb-231 breast cancer cells in 100 μl phosphate-buffered saline (pbs; ph 7.4) plus 100 μl of matrigel (bd biosciences, san jose, ca, usa) injected into their left second mammary fat pads.
PubMedID- 24776983 severe combined immunodeficiency (scid) mice, which are deficient in t and b cells, appear to be resistant to the enteropathic effects of seb.
PubMedID- 24318653 Next, we utilized the severe combined immunodeficiency (nod-scid) mouse model and injected lymphoma cells with or without stromal cells and observed a more robust growth of tumor in mice receiving hk and lymphoma cells[7].
PubMedID- 23646141 Briefly, nod/scid (non-obese diabetic – severe combined immunodeficiency) female mice had mda-mb-231 breast cancer cells in 100 ml phosphate buffered saline (pbs; ph 7.4) plus 100 ml of matrigel (bd biosciences, san jose, ca, usa) injected into their left second mammary fat pads.
PubMedID- 23300452 Moreover, consistent with the lack of enhanced susceptibility of patients with acquired immunodeficiency syndrome, severe combined immunodeficiency, x-linked agammaglobulinemia, and common variable immunodeficiency to invasive candidiasis, t and b lymphocytes are dispensable for host defense in the mouse model [26].
PubMedID- 26425655 Jak3 and/or stat5b mutations that cause severe combined immunodeficiency (scid) syndrome in humans) are prone to severe infections.
PubMedID- 23202463 Used the severe combined immunodeficiency disorder (scid) mice transgenic for urokinase plasminogen activator (upa) under control of the albumin (alb) promoter that transplanted with human hepatocyte model (scid/alb-upa chimeric mice model) for hcv infection to test whether hcv can activates er stress in vivo [53].
PubMedID- 25346775 Adoptive immunization of severe combined immunodeficiency mice with t cells from 2d2 live-attenuated bpm mutant-immunized mice resulted in increased survival compared to naïve t cell recipients.
PubMedID- 22941246 Adult nonobese diabetic/severe combined immunodeficiency interleukin 2 receptor gamma chain knock out (nsg) mice (jackson laboratories, bar harbor, me), were used for in vivo experiments according to protocols approved by the institutional animal care and use committee of university of california los angeles.
PubMedID- 20824134 Nonobese diabetic/severe combined immunodeficiency (nod/scid) mice were bred and maintained in the johns hopkins animal core facility.
PubMedID- 21701688 Briefly, female, severe combined immunodeficiency (scid) mice (17–20 g, 4–6 weeks old) were bred in house and maintained throughout in specific pathogen-free (spf) isolators.
PubMedID- 21544519 severe combined immunodeficiency (scid) is part of the differential diagnosis of cd40l deficiency (van der burg m, gennery a, the expanding spectrum of severe combined immunodeficiency, ejp in press), but in contrast to most cases of scid, analysis of lymphocyte subpopulation in pad patients will show normal t cell counts.
PubMedID- 24389287 Nod/scid (non-obese diabetic/severe combined immunodeficiency) mice were injected with 1.5x106 sk-n-as cells in pbs and matrigel basement membrane matrix (100μm) (bd biosciences, franklin lakes, nj usa) subcutaneously in the left flank.
PubMedID- 24740260 Cells with deletion of any of these nhej components show severe combined immunodeficiency (scid) due to defects in assembling variable (diverse) joining (v(d)j) segments of antigens, genetic instability and hypersensitivity to dsb inducing agents [5]–[9].
PubMedID- 20979627 Nhej defects lead to severe combined immunodeficiency (scid) and lymphoid cancer predisposition in both mice and humans.
PubMedID- 24466340 Lastly, mutations of dna-pkcs cause severe combined immunodeficiency in mice [17], [18], [19], [20].
PubMedID- 26022250 The derivations of nude and severe combined immunodeficiency (scid) mice, which are widely used for xenotransplantation, were milestones in the development of immunodeficient mice.
PubMedID- 23898329 Due to the severe combined immunodeficiency background, these mice are deficient in functional mature t and b cells.
PubMedID- 23264026 severe combined immunodeficiency (scid), characterized by extremely low or absent t cell production, defective t cell function and absent antibody responses, can be caused by defects in any of several genes and if untreated leads to early death due to infections [1, 2].
PubMedID- 21379384 severe combined immunodeficiency (scid) mice, first successfully established in 1983 by bosma et al.
PubMedID- 21170549 An effective diagnostic strategy should be able to quickly identify pids with rapidly fatal complications like severe combined immunodeficiency (scid) and to limit diagnostic delay in children with pids with a more protracted course like common variable immunodeficiency disorders (cvids), who may develop bronchiectasis with pulmonary disability [15] and decreased life span if treatment is delayed.
PubMedID- 24728301 The 6- to 8-week old male nod-scid mice (non-obese diabetes severe combined immunodeficiency mice) were used to evaluate the in vivo meta-static behavior of tumor cells.
PubMedID- 23831714 We also performed fluorescence microscopy ivfc on severe combined immunodeficiency (scid) mice in parallel (as has been described in detail previously)5 to compare and validate the msc clearance kinetics measured with the dffc prototype.
PubMedID- 25972190 Severe acquired immunodeficiencies (e.g., uncontrolled hiv or severe combined immunodeficiency) or genetic defects in the il-23/th17 pathway can increase patients' susceptibility to a number of syndromes that are all associated with chronic or recurrent mucosal or skin infections with candida albicans (table 1; puel et al., 2010b; hanna and etzioni, 2011; huppler et al., 2012).
PubMedID- 25283340 severe combined immunodeficiency (scid) mice were bred and maintained in the animal facility of the department of dermatology (university of debrecen) in accordance with the animal-welfare ordinance.
PubMedID- 21589826 Patients with a complete absence of the thymus (“complete” digeorge syndrome) exhibit severe t-cell immunodeficiency with a severe combined immunodeficiency phenotype requiring immune reconstitution by bone marrow transplantation or thymic transplantation.5–7 however, “complete” digeorge syndrome accounts for <1% of patients.8,9 the majority of patients with 22q11.2 ds and immune defects exhibit mild to moderate deficits in t-cell numbers (so-called “partial” digeorge syndrome).
PubMedID- 24592361 [91011] these vectors are used for the treatment of a number of diseases such as β-thalassemia, hemophilia, severe combined immunodeficiency (scid), cystic fibrosis, and muscular and neurodegenerative diseases in animal models.
PubMedID- 20668627 Ada deficiency is the major metabolic cause of severe combined immunodeficiency disease.31,32 ada is important for the development of the immune system in humans.
PubMedID- 25550434 Female severe combined immunodeficiency (scid) mice (charles river laboratory) in the +dox group were switched to 400 ppm doxycycline diet (harlan) 3 days prior to implant, while animals in the −dox group were maintained on standard mouse diet.
PubMedID- 22469436 A 12-year-old girl with severe combined immunodeficiency was admitted to the freiburg university medical center, germany, in november 2009 for treatment of progressive respiratory problems and cytomegalovirus (cmv) disease.
PubMedID- 21573181 Immunodeficient non obese diabetous/shi-severe combined immunodeficiency/interleukin-2rγnull (nog) mice were injected with pm7 and ags mixed at a ratio of 8∶1 (2 250 000 and 375 000 cells, respectively) resuspended in 200 µl of 7 mg/ml matrigel (bd biosciences) in ice cold pbs.
PubMedID- 24670249 Female nod-scid (non-obese diabetic-severe combined immunodeficiency; tzu chi university, hualien, taiwan mice were purchased from jackson lab, bar harbor, me, usa) and housed under specific pathogen-free conditions in the animal center of the institute of cellular and organismic biology of sinica.
PubMedID- 22355452 severe combined immunodeficiency (scid) mice lack both humoral and cellular responses due to deficiencies in b and t cell development.

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