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eRAM

encyclopedia of Rare Disease Annotation for Precision Medicine




Disease severe combined immunodeficiency
Comorbidity C0085110|severe combined immunodefic
Sentences 113
PubMedID- 20020426 Adult male severe combined immunodeficient (scid) mice (c.b-17/icrhsd-scid) were purchased (harlan, indianapolis, in, http://www.harlan.com).
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- 24564963 The 21- to 23-week-old female severe combined immunodeficient (scid/beige) or dba/1 mice (five per group) were grown in our animal facilities.
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- 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- 24141776 Six to eight-week old female non-obese diabetic/severe combined immunodeficient (nod/scid) mice were obtained from the fhcrc core center of excellence in hematology (dk-56465) and housed under pathogen-free conditions at the fhcrc comparative medicine shared resource.
PubMedID- 24839982 Male cb-17 non-obese diabetic/severe combined immunodeficient (nod.scid) mice (6- to 8-weeks old; harlan laboratories, inc., indianapolis, in, us) were housed and monitored in our animal research facility.
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- 20465788 The spectrum of t cell defects is broad, from severe combined immunodeficiency to signaling defects to specific defects in lymphocyte apoptosis.
PubMedID- 20824134 Nonobese diabetic/severe combined immunodeficiency (nod/scid) mice were bred and maintained in the johns hopkins animal core facility.
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- 24062996 The use of athymic nude mice and severe combined immunodeficient mice serve to avoid graft rejection but the former mice develop new histological changes not seen in psoriasis while the latter mice continue to manifest rejection of the xenogeneic tissue due to presence of nk cells [35].
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- 24039951 Six-week-old female nonobese diabetic/severe combined immunodeficient (nod/scid) mice (jackson laboratory) were implanted with 5.0×103 sorted aldh-positive sum-149 cells in a 30 µl 50% matrigel (bd biosciences, cb-40230a) solution (1∶1 dilution of matrigel with ham’s f-12 medium) in the fourth inguinal mammary fat pad.
PubMedID- 24587095 severe combined immunodeficient (scid) mice (male, 5-week-old) were purchased from jackson laboratory and maintained in m. d. anderson’s animal facilities.
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- 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- 25962155 All animal studies with female severe combined immunodeficient (scid) mice (c.b.-17/icrhsd-prkdcscid lystbg; harlan laboratories) were conducted with approval from, and in accordance with, the mayo clinic institutional animal care and use committee, accredited by the american association of laboratory animal care, which meet or exceed the standards set by the u.s. department of agriculture animal welfare act, public health service policy on humane care and use of animals, and the nih guide on laboratory animal welfare.
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- 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- 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- 20979627 Nhej defects lead to severe combined immunodeficiency (scid) and lymphoid cancer predisposition in both mice and humans.
PubMedID- 24375628 Six-week-old female nonobese diabetic/severe combined immunodeficient (nod/scid) mice (jackson laboratory, bar harbor, me, usa) were implanted with 2.5 × 106 cells in 50 μl of a 1:1 dilution of matrigel (cb-40230a; bd biosciences, san jose, ca, usa) and dmem medium, in the fourth inguinal mfp.
PubMedID- 24069355 Thus, for the first time we present t-b-nk+ severe combined immunodeficiency (scid) phenotype after spontaneously occurring modification of artemis gene in mice.
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- 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- 24198970 Hyper-ige and wiskott-aldrich syndromes, cd40l deficiency, severe combined immunodeficiency, x-linked agammaglobulinemia, transient hypogammaglobulinemia of infancy, and chronic granulomatous disease were diagnosed in these children.
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- 24216290 Female severe combined immunodeficient (scid) mice at 4 to 6 weeks of age were used in the orthotopic tumor-xenograft model.
PubMedID- 25942583 Sixteen severe combined immunodeficiency (cb17/scid) mice obtained from charles river (wilmington, ma) were housed in an animal care facility and held for 10 days to acclimatize.
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- 24116047 Female non-obese diabetic/severe combined immunodeficiency (nod/scid) mice (5 week-old) were purchased (clea japan, inc., osaka, japan), and housed in laminar-flow cabinets under specific pathogen-free conditions.
PubMedID- 21637808 Four to six week old female c3h/he, c57bl/6 and severe combined immunodeficient c57bl/b6.c-prkdcscid (scid) mice were obtained from the jackson laboratory, bar harbor, me, and maintained at uc davis in isolator cages under conventional housing conditions.
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- 24772353 severe combined immunodeficient (scid) mice lacking either t and b lymphocytes or nude mice lacking only t cells have impaired proliferation and neurogenesis in normal and ee housing compared to wild-type mice [155], as well as impaired performance in the water maze [156].
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- 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- 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- 20226009 Scid (severe combined immunodeficiency) mice were maintained in a specific pathogen-free environment in compliance with institutional policy and all animal procedures were previously approved by the iacuc (institutional animal care and use committee) at taipei medical university.
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- 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- 23226053 This is true of patients with ataxia telangiectasia (at), ataxia telangiectasia-like disorder, severe combined immunodeficiency, ligase iv syndrome, rothmund–thompson syndrome, seckel syndrome, werner syndrome, nijmegen breakage syndrome, all due to defective repair of double-strand breaks (dsbs)5 or stalled replication forks.6 it is also true of patients with fanconi anemia caused by defective repair of dna interstrand crosslinks (icls) and patients with xeroderma pigmentosum due to a defect in nucleotide excision repair (ner) of helix-distorting dna adducts.7,8 since nsclc and hnscc are treated with cisplatin and radiation therapy, it is logical to predict that patients with reduced dsb repair, single-strand break (ssb) repair, icl repair, or ner due to polymorphisms affecting the expression or function of dna repair proteins might be most responsive to dna damaging agents.
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- 20706692 Likewise, reconstitution of severe combined immunodeficient mice or recombinase activating gene 1 deficient mice with naïve cd4+cd45rbhi positive t cells results in significant intestinal inflammation and barrier disruption again due to dysregulation of lymphocyte responses [32].
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- 22843486 severe combined immunodeficient (scid) mice (seven per group) were grafted with human synovium and skin on either side of the animal subcutaneously in a dorsal position distal to the shoulder joints.
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.
PubMedID- 21379384 severe combined immunodeficiency (scid) mice, first successfully established in 1983 by bosma et al.
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- 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.

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