004088 B6.Cg- Foxp3 sf /J Scurfy mice have defective T cell tolerance leading to an X-linked lymphoproliferative disease that parallels the X-linked autoimmunity-allergic disregulation syndrome (XLAAD) in humans.

Apr 11, 2014 · Scurfy mice are characterized by scaly and ruffled skin, reddened eyes, enlargement of the spleen and lymph nodes, and premature death (usually at approximately 3 weeks after birth).

Scurfy mice are deficient in regulatory T cells (Tregs), develop a severe, generalized autoimmune disorder that can affect almost every organ and die at an early age. Some of these manifestations resemble those found in systemic lupus erythematosus (SLE).

Scurfy mice have a complete deficiency of functional regulatory T cells (Treg) due to a frameshift mutation in the Foxp3 gene. The impaired immune homeostasis results in a lethal lymphoproliferative disorder affecting multiple organs, including the liver.

Due to a mutation in the Foxp3 transcription factor, Scurfy mice lack regulatory T-cells that maintain self-tolerance of the immune system. They develop multi-organ inflammation (MOI) and die around four weeks old. The affected organs are skin, tail, lungs and liver.

Apr 23, 2019 · Scurfy mice are characterized by a complete functional deficiency of regulatory T cells (Treg) due to an X-linked frameshift mutation in the Foxp3 gene, resulting in an impairment of peripheral tolerance in hemizygous males.

Mar 1, 1999 · Mice hemizygous (X sf /Y) for the X-linked mutation scurfy (sf) develop a severe and rapidly fatal lymphoproliferative disease mediated by CD4 + CD8 − T lymphocytes. We have undertaken phenotypic and functional studies to more accurately identify the immunologic pathway (s) affected by this important mutation.

Apr 24, 2019 · U1-ribonucleoprotein (U1RNP) was found to be the most common target antigen recognized by autoantibodies in scurfy mice. Additionally, scurfy mice exhibited a mild myositis with histological characteristics similar to polymyositis/dermatomyositis.

Scurfy (sf) is an X-linked recessive mouse mutant resulting in lethality in hemizygous males 16–25 days after birth, and is characterized by overproliferation of CD4+CD8– T...

Apr 29, 2021 · Scurfy mice present a spontaneous Foxp3 mutation. Within the first 10 days of life, these mice develop a fatal disease with organ-specific autoimmunity 10 that reproduces the most severe IPEX syndrome.