関: SCID mouse、SCID-hu mice、SCID-hu mouse、severe combined immunodeficient mice、severe combined immunodeficient mouse

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ミ(全音階の第3音)
mouseの複数形

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2016/09/08 04:22:53」(JST)

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The severe combined immunodeficiency (SCID) is a severe immunodeficiency genetic disorder that is characterized by the complete inability of the adaptive immune system to mount, coordinate, and sustain an appropriate immune response, usually due to absent or atypical T and B lymphocytes. In humans, SCID is colloquially known as "bubble boy" disease, as victims may require complete clinical isolation to prevent lethal infection from environmental microbes.

Several forms of SCID occur in animal species. Not all forms of SCID have the same cause; different genes and modes of inheritance have been implicated in different species.

Horses

Equine SCID is an autosomal recessive disorder that affects the Arabian horse. Similar to the "bubble boy" condition in humans, an affected foal is born with no immune system, and thus generally dies of an opportunistic infection, usually within the first four to six months of life. There is a DNA test that can detect healthy horses who are carriers of the gene causing SCID, thus testing and careful, planned matings can now eliminate the possibility of an affected foal ever being born.^[1]^[2]^[3]

SCID is one of six genetic diseases known to affect horses of Arabian bloodlines, and the only one of the six for which there is a DNA test to determine if a given horse is a carrier of the allele.^[4] There are other genetic diseases that affect other horse breeds,^[5] and horses of part-Arabian bloodlines can be carriers of SCID.

Unlike SCID in humans, which can be treated, for horses, to date, the condition remains a fatal disease.^[6] When a horse is heterozygous for the gene, it is a carrier, but perfectly healthy and has no symptoms at all. If two carriers are bred together, however, classic Mendelian genetics indicate that there is a 50% chance of any given mating producing a foal that is a carrier heterozygous for the gene, and a 25% risk of producing a foal affected by the disease. If a horse is found to carry the gene, the breeder can choose to geld a male or spay a female horse so that they cannot reproduce, or they can choose to breed the known carrier only to horses that have been tested and found to be "clear" of the gene. In either case, careful breeding practices can avoid ever producing an SCID-affected foal.

Dogs

There are two known types of SCID in dogs, an X chromosome-linked form that is very similar to X-SCID in humans,^[7] and an autosomal recessive form that is similar to the disease in Arabian horses and SCID mice.^[8]

X-SCID in dogs is seen in Basset Hounds and Cardigan Welsh Corgis. Because it is an X-linked disease, females are carriers only and disease is seen in males exclusively. It is caused by a mutation in the gene for the cytokine receptor common gamma chain.^[7] Recurring infections are seen and affected animals usually do not live beyond three to four months. Characteristics include a poorly developed thymus gland, decreased T-lymphocytes and IgG, absent IgA, and normal quantities of IgM.^[9] A common cause of death is canine distemper, which develops following vaccination with a modified live distemper virus vaccine.^[10] Due to its similarity to X-SCID in humans, breeding colonies of affected dogs have been created in order to study the disease and test treatments, particularly bone marrow transplantation and gene therapy.^[11]

The autosomal recessive form of SCID has been identified in one line of Jack Russell Terriers. It is caused by a loss of DNA protein kinase, which leads to faulty V(D)J recombination. V(D)J recombination is necessary for recognition of a diverse range of antigens from bacteria, viruses, and parasites. It is characterized by nonfunctional T and B-lymphocytes and a complete lack of gammaglobulins.^[10] Death is secondary to infection. Differences between this disease and the form found in Bassets and Corgis include a complete lack of IgM and the presence of the disease in females.

Mice

A close-up of white Severe combined immunodeficiency (SCID) mouse held by a human hand.

SCID mice are routinely used as model organisms for research into the basic biology of the immune system, cell transplantation strategies, and the effects of disease on mammalian systems. They have been extensively used as hosts for normal and malignant tissue transplants. In addition, they are useful for testing the safety of new vaccines or therapeutic agents in immunocompromised individuals.

The condition is due to a rare recessive mutation on Chromosome 16 responsible for deficient activity of an enzyme involved in DNA repair (Prkdc or "protein kinase, DNA activated, catalytic polypeptide"). Because V(D)J recombination does not occur, the humoral and cellular immune systems fail to mature. SCID mice, therefore, present with impaired ability to make T or B lymphocytes, or activate some components of the complement system, and cannot efficiently fight infections, nor reject tumors and transplants.

By crossing SCID mice with mice carrying mutations in related genes, such as interleukin-2Rgamma,^[12] more efficient immunocompromised strains can be created to further aid research. The degree to which the various components of the immune system are compromised varies according to what other mutations the mice carry along with the SCID mutation.

References

^ "SCID in Arabian Horses"
^ Parkinson, Mary Jane. "SCID: An Update." from Arabian Horse World, March, 1998
^ "The New DNA Test for Severe Combined Immunodeficiency (SCID) in Arabian Horses"
^ AHA Equine Stress, Research and Education Committee. "Caution:Knowledge." Modern Arabian Horse, August/September 2007, pp. 100-105. Online version at http://modernarabianhorse.epubxpress.com/?site=1
^ Other breeds known to have some individuals with genetic conditions include the American Quarter Horse, American Paint Horse, American Saddlebred, Appaloosa, Miniature horse, and Belgian.
^ FOAL.org, an organization promoting research into genetic lethal diseases in horse
^ ^a ^b Henthorn PS, Somberg RL, Fimiani VM, Puck JM, Patterson DF, Felsburg PJ (1994). "IL-2R gamma gene microdeletion demonstrates that canine X-linked severe combined immunodeficiency is a homologue of the human disease" (abstract only). Genomics. 23 (1): 69–74. doi:10.1006/geno.1994.1460. PMID 7829104.
^ Bell TG, Butler KL, Sill HB, Stickle JE, Ramos-Vara JA, Dark MJ (2002). "Autosomal recessive severe combined immunodeficiency of Jack Russell terriers". J. Vet. Diagn. Invest. 14 (3): 194–204. doi:10.1177/104063870201400302. PMID 12033674.
^ Perryman LE (2004). "Molecular pathology of severe combined immunodeficiency in mice, horses, and dogs". Vet. Pathol. 41 (2): 95–100. doi:10.1354/vp.41-2-95. PMID 15017021.
^ ^a ^b Ettinger, Stephen J.; Feldman, Edward C. (2005). Textbook of Veterinary Internal Medicine (6th ed.). W.B. Saunders Company. ISBN 1-4160-0110-7.
^ Ting-De Ravin SS, Kennedy DR, Naumann N, et al. (2006). "Correction of canine X-linked severe combined immunodeficiency by in vivo retroviral gene therapy". Blood. 107 (8): 3091–7. doi:10.1182/blood-2005-10-4057. PMC 1895747. PMID 16384923.
^ Ito M, Hiramatsu H, Kobayashi K, et al. (November 2002). "NOD/SCID/gamma(c)(null) mouse: an excellent recipient mouse model for engraftment of human cells". Blood. 100 (9): 3175–82. doi:10.1182/blood-2001-12-0207. PMID 12384415.

UpToDate Contents

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1. 重度複合型免疫不全（SCID）：概要 severe combined immunodeficiency scid an overview
2. 重度複合型免疫不全（SCID）：特定の欠損 severe combined immunodeficiency scid specific defects
3. JAK3欠損を伴う重度複合型免疫不全（SCID） severe combined immunodeficiency scid with jak3 deficiency
4. T-B-NK+ SCID：病因および遺伝学 t b nk scid pathogenesis and genetics
5. T-B-NK+ SCID：臨床症状、診断、および治療 t b nk scid clinical manifestations diagnosis and treatment

English Journal

Impact of clinically tested NEP/ACE inhibitors on tumor uptake of [(111)In-DOTA]MG11-first estimates for clinical translation.

Kaloudi A1, Nock BA2, Lymperis E3, Valkema R4, Krenning EP5, de Jong M6,7, Maina T8.
EJNMMI research.EJNMMI Res.2016 Dec;6(1):15. doi: 10.1186/s13550-015-0158-3. Epub 2016 Feb 16.
BACKGROUND: We have recently shown that treatment of mice with the neutral endopeptidase (NEP) inhibitor phosphoramidon (PA) improves the bioavailability and tumor uptake of biodegradable radiopeptides. For the truncated gastrin radiotracer [(111)In-DOTA]MG11 ([(DOTA)DGlu(10)]gastrin(10-17)), this m
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Novel triterpenoid AECHL-1 induces apoptosis in breast cancer cells by perturbing the mitochondria-endoplasmic reticulum interactions and targeting diverse apoptotic pathways.

Sawant MA1, Dasgupta A1, Lavhale MS2, Sitasawad SL3.
Biochimica et biophysica acta.Biochim Biophys Acta.2016 Jun;1860(6):1056-70. doi: 10.1016/j.bbagen.2016.02.002. Epub 2016 Feb 3.
BACKGROUND: The modus operandi for an anti-cancer drug must allow for an efficient discrimination system between tumorigenic and non-tumorigenic cells. Targeting ER stress and mitochondrial function in cancer cells appears to be a suitable option, as these processes are dysregulated in tumor cells.
PMID 26851676

Lysyl oxidase like-4 monoclonal antibody demonstrates therapeutic effect against head and neck squamous cell carcinoma cells and xenografts.

Görögh T1, Quabius ES1,2, Heidebrecht H2, Nagy A1, Muffels T1, Haag J3, Ambrosch P1, Hoffmann M1.
International journal of cancer.Int J Cancer.2016 May 15;138(10):2529-38. doi: 10.1002/ijc.29986. Epub 2016 Jan 25.
A new member of the lysyl oxidase (LOX) family, lysyl oxidase-like 4 (LOXL4), is overexpressed in head and neck squamous cell carcinoma (HNSCC) compared to normal squamous epithelium. A monoclonal antibody (mAb) derived from fusion of Balb/c mouse splenocytes immunized with LOXL4 specific peptide wa
PMID 26756583

Japanese Journal

Proliferation of functional human natural killer cells with anti-HIV-1 activity in NOD/SCID/Jak3[null] mice

Microbiology and immunology 60(2), 106-113, 2016-02
NAID 40020747678

Osteopontin-integrin interaction as a novel molecular target for antibody-mediated immunotherapy in adult T-cell leukemia

Retrovirology 12, 99, 2015-11-24
NAID 120005690998

Absence of lactobacilli containing glycolipids with the α-galactose epitope and the enhanced fucosylation of a receptor glycolipid GA1 in the digestive tracts of immune-deficient scid mice

The journal of biochemistry 158(1), 73-82, 2015-07
NAID 40020522527

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リンク元	「severe combined immunodeficient mice」「重症複合免疫不全マウス」「SCID-hu mice」「SCID-hu mouse」「SCIDマウス」
関連記事	「mi」「SC」「SCID」