A severe combined immunodeficiency mutation in the mouse

@article{Bosma1983ASC,
  title={A severe combined immunodeficiency mutation in the mouse},
  author={Gayle C. Bosma and R. Philip Custer and Melvin J. Bosma},
  journal={Nature},
  year={1983},
  volume={301},
  pages={527-530}
}
The most debilitating human lymphoid deficiency disease, known as severe combined immunodeficiency (SCID), impairs the differentiation of both T and B lymphocytes1–7. Affected infants are highly susceptible to recurring infections of viruses, fungi and bacteria and invariably die within 2yr of birth. Inheritance of this congenital syndrome may show X-linked8,9 or autosomal recessive control1,2,9. To date autosomal recessive inheritance of SCID has been observed in Arabian foals10 which… 
Human Humoral Immunity in SCID Mice
TLDR
Characteristics of SCID mice reconstituted with human lymphoid cells, mainly hu-PBL-SCID mice, are described, with emphasis on parameters which may be relevant to humoral immunity.
Metabolic defects in severe combined immunodeficiency in man and animals.
  • N. Magnuson, L. Perryman
  • Biology, Medicine
    Comparative biochemistry and physiology. B, Comparative biochemistry
  • 1986
TLDR
Two animal models for SCID now exist and neither of these models is associated with ADA deficiency, which indicates that SCID is a heterogeneous group of diseases that are clinically similar but are caused by quite different biochemical abnormalities.
X-linked severe combined immunodeficiency in the dog.
TLDR
The disease in the dog is characterized by growth retardation and increased susceptibility to bacterial and viral infections in young pups, supported by the failure of peripheral lymphocytes to produce IgG or IgA plaque-forming cells in response to polyclonal activation.
Equine Severe Combined Immunodeficiency Av
Severe Combined Immunodeficiency (SCID) is a disease with a genetic background in Arabian horses and crossbreeds with Arabians. The disease has been known and documented since the 1970’s and was
Equine severe combined immunodeficiency
TLDR
The question whether heterozygotes should be allowed in breeding or not should be decided while considering the population size and the frequency of carriers, because the total population of Arabian horses in Sweden is large enough to exclude carriers from breeding.
Pneumocystis carinii pneumonia in scid/scid mice.
TLDR
The use of scid/scid mice as models to study development of human lymphoid cells following transplantation of human fetal tissues and to study interactions occuring during the course of infection of human cells with human immunodeficiency virus (HIV) is described.
Spontaneous development of neoplasms in severe combined immunodeficient mice
  • R. Samuel
  • Medicine
    SAGE open medical case reports
  • 2015
TLDR
The differential diagnosis of such an atypical lymphoid infiltrate includes ‘leaky’ severe combined immunodeficient mice, thymic lymphoma and acute leukaemia.
Molecular Pathology of Severe Combined Immunodeficiency in Mice, Horses, and Dogs
TLDR
Two molecular mechanisms account for SCID in dogs: Jack Russell Terriers have a mutation within the DNA-PKcs gene, whereas Cardigan Welsh Corgi and Basset Hound have different defects in the gene encoding the γ chain that is common to the receptors for IL-2.
SCID Mice as a Model for Human Leukemias
TLDR
This chapter deals exclusively with the engraftment of human leukemias in this model system and the usefulness of the SCID mouse for studying murine and human hematopoiesis.
Severe Combined Immunodeficiencies
TLDR
The clinical and immunological phenotypes and recent insights into the pathophysiology of clinical manifestations of immune dysregulation associated with immunodeficiency are described.
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