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Pediatric Disease Annotations & Medicines




Disease severe combined immunodeficiency
Phenotype |severe combined immunodefic
Sentences 174
PubMedID- 20465788 severe combined immunodeficiency (scid) describes a heterogeneous group of genetically determined conditions which result in lymphopenia and hypogammaglobulinemia, with inability to fight infection and early death.
PubMedID- 23815869 severe combined immunodeficient (scid) male mice, 4 to 6 weeks of age (charles river laboratories, wilmington, massachusetts), were housed in a barrier facility and maintained on a normal diet ad libitum.
PubMedID- 23833664 The nonobese diabetic/severe combined immunodeficient (nod/scid) mice were purchased from shanghai slac laboratory animal co. ltd. (shanghai, china) and maintained in micro isolator cages.
PubMedID- 22282699 When severe combined immunodeficient mice injected with ba/f3 cells expressing bcr-abl isoforms and the firefly luciferase gene, a decrease in tumor burden measured by bioluminescence imaging was observed in das-treated mice harboring the wild-type bcr-abl and m351t, but not the t315i mutant.13 these experiments laid the ground for a first in human clinical trial.
PubMedID- 22567029 Children with severe combined immunodeficiency can develop bcgosis after bcg immunization as a result of their deficient immune system, which clearly illustrates that vaccinations should only be performed in immunocompetent individuals [20].
PubMedID- 20158571 Eight-week-old severe combined immunodeficiency (scid) mice were treated with streptozotocin (stz, 200 mg/kg, sigma) freshly dissolved in 0.025 m sodium citrate (ph 4.0).
PubMedID- 23343349 Ak 2 mutations cause reticular dysgenesis, the most severe combined immunodeficiency in humans [48].
PubMedID- 20138044 Akr, balb/c (harlan, uk), and severe combined immunodeficient (scid) mice were maintained in the biological services unit at manchester university.
PubMedID- 24683542 Male cb-17 severe combined immunodeficient (scid) mice (6- to 8-week old; harlan laboratories, inc., indianapolis) were housed and monitored in our animal research facility.
PubMedID- 20547828 Affected patients manifest with symptoms of severe combined immunodeficiency (scid), including an increased occurrence of life-threatening infections, failure to thrive, and, in particular, autoimmune-like clinical features including early-onset severe erythrodermia, alopecia, hepato-splenomegaly, and lymphadenopathy (omenn, 1965; ochs et al., 1974).
PubMedID- 26495209 severe combined immunodeficiency beige mice (charles river laboratories, lyon, france) were used for in vivo fat grafting experiments.
PubMedID- 20927287 [335659] furthermore, severe combined immunodeficient (scid) mice, which lack t cells but have normal nk function, are able to contain l. major parasites in the draining lymph nodes, arguing for the existence of a t cell-independent mechanism to limit parasite spread.
PubMedID- 21994645 Approval of adagen® (pegademase) for the treatment of severe combined immunodeficiency disease (scid) by the u.s. fda in the early 1990s illustrated the potential for pegylation to significantly impact modern therapeutics.
PubMedID- 23758766 We used a severe combined immunodeficiency (scid) hiv encephalitis (hive) mouse model in which intracranial injection of clade b hiv-infected mdms leads to neuropathology and neurocognitive defects that parallel those found in had patients [20,21].
PubMedID- 24723986 The xenograft tumor volume of the severe combined immunodeficiency (scid) mice after 20 day treatment with the plga nanoparticles formulation combined with 3-methyladenine or chloroquine are found to be only about a half in comparison with the plga nanoparticles formulation only 58. thus, the incorporation of autophagy inhibitor in future theranostic platform, can improve the co-delivery of diagnostic and therapeutic agent.
PubMedID- 20946682 Male nonobese diabetic-severe combined immunodeficient (nod-scid) mice (6 weeks old) were obtained from jackson laboratory (bar harbor, me), and maintained and cared in accordance with the guide for the care and use of laboratory animals.
PubMedID- 23574525 On the other hand, genetic deficiency of jak3 does not lead to lethality but causes severe combined immunodeficiency (scid) in humans [45,46] and a corresponding phenotype with severe lymphocyte developmental defects in mice [47,48].
PubMedID- 20824134 Nonobese diabetic/severe combined immunodeficiency (nod/scid) mice were bred and maintained in the johns hopkins animal core facility.
PubMedID- 20360964 Eight-week-old male severe combined immunodeficient (scid) mice (n = 14) were purchased from charles river laboratories (wilmington, ma, usa) and housed in specific-pathogen-free conditions.
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- 25798061 Four- to six-week-old female balb/c severe combined immunodeficient (scid) were obtained from frederick cancer research and development center (national cancer institute) and housed in a specific pathogen-free animal facility.
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- 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- 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- 21379384 severe combined immunodeficiency (scid) mice, first successfully established in 1983 by bosma et al.
PubMedID- 25773070 Non obese diabetic/severe combined immunodeficient (nod/scid) janus kinase 3 knockout (noj) mice26 were anesthetized by isoflurane and placed in the supine position.
PubMedID- 21067584 By means of a non-obese diabetic/severe combined immunodeficiency disease (nod/scid) xenotransplant assay in combination with specific cell surface markers (cd44+cd24-/low), cscs were enriched from metastatic and primary breast tumors and were shown to have the ability to reestablish tumor heterogeneity after transplantation [1].
PubMedID- 20979627 Nhej defects lead to severe combined immunodeficiency (scid) and lymphoid cancer predisposition in both mice and humans.
PubMedID- 23898329 Due to the severe combined immunodeficiency background, these mice are deficient in functional mature t and b cells.
PubMedID- 24198507 severe combined immunodeficiencies (scid) are a set of diseases caused by monogenic disorders that impair t-cell development and, depending on the gene implicated, can be associated with faults in the development of other hematopoietic lineages.
PubMedID- 26448762 Nonobese, diabetic/severe combined immunodeficient (nod/scid) immunocompromised mice (5-week-old female mice) were purchased from charles river laboratories international, inc. (wilmington, ma) and maintained in microisolator cages.
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- 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- 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- 26442241 For example, prior to 2005, severe combined immunodeficiency (scid) could not be included in newborn screening as there was no method of testing for the condition using a dried bloodspot.
PubMedID- 22251838 The non-obese diabetic/severe combined immunodeficient (nod/scid) mice expressing enhanced-green fluorescent protein (egfp), combined with dsred transfected tumor cells enables studies of tumor-stroma cell interactions, both in situ and ex vivo [6].
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- 23298290 Hairless (severe combined immunodeficiency) scid or athymic nu/nu (or nude) female mice, 5 to 6 wk old (charles river laboratories, inc., wilmington, ma) were maintained under pathogen-free conditions in hepa-filtered cages under controlled light (12 h light and dark cycle), temperature (22-24°c), and humidity (25%).
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- 23738757 Four to five weeks old non-obese diabetic/severe combined immunodeficiency (nod/scid) male mice were used in all experiments.
PubMedID- 21233838 The development of the scid (severe combined immunodeficiency)-hu model has been an important advance, as it was the first model to recapitulate a hubmm in mice.4, 5, 6 however, although the scid-hu system remains a highly relevant model for preclinical investigation, it does have important limitations: (i) restricted availability of human fetal bone chips; (ii) the allogeneic nature of the fetal bm milieu versus mm cells; and (iii) the significant heterogeneity of implanted human bone chips, collected from different individuals at different gestational age, that may produce experimental variability.
PubMedID- 20617166 As a result of the recent development of a vzv cosmid system and of the severe combined immunodeficient mouse model with xenografts of human tissue (scid-hu), many viral orfs have been investigated in both biochemical and functional studies, shedding light upon several vzv gene functions [16]–[18].
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- 25806119 Non-obese diabetic/severe combined immunodeficient (nod/scid) female mice 8-10 weeks of age were anesthetized and received a flank subcutaneous injection of 1×106 cultured l0 gbm cells in 200 µl of medium and 100 µl of matrigel (bd).
PubMedID- 21510863 severe combined immunodeficient (scid) mice were inoculated with trastuzumab-resistant jimt-1 cells to investigate the tumour inhibitory effect of t-dm1 in vivo.
PubMedID- 25859981 Male severe combined immunodeficient (scid) mice were included in the study at 4–6 weeks of age.
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- 23555725 The animals used here were transgenic severe combined immunodeficient (scid) mice that carried additional copies of the urokinase plasminogen activator-encoding gene, resulting in the apoptosis of endogenous mouse hepatocytes, which then were replaced with human hepatocytes.
PubMedID- 23570619 severe combined immunodeficient mice carrying hela tumor xenografts were treated in groups of six including phosphate-buffered saline control, blank tpgs-b-(pcl-ran-pga) nanoparticles, blank tpgs-b-(pcl-ran-pga)/pei nanoparticles, and three types of gene nanoparticles.
PubMedID- 21819554 severe combined immunodeficient mice were injected in 0.2 ml volume s.c. on the right flank and observed daily for tumor appearance.

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