Fatal lymphoreticular disease in the scurfy (sf) mouse requires T cells that mature in a sf thymic environment: Potential model for thymic education

Journal Article · Mon Jul 01 00:00:00 EDT 1991 · Proceedings of the National Academy of Sciences of the United States of America; (United States)

DOI:https://doi.org/10.1073/pnas.88.13.5528· OSTI ID:6096505

Godfrey, V L; Rinchik, E M; Russell, L B ^[1]; Wilkinson, J E ^[2]

---

1. Oak Ridge National Lab., Oak Ridge, TN (United States)
2. Univ. of Tennessee, Knoxville (United States)

Characteristic lesions in mice hemi- or homozygous for the X-linked mutation scurfy (sf) include lymphohistiocytic proliferation in the skin and lymphoid organs, Coombs' test-positive anemia, hypergammaglobulinemia, and death by 24 days of age. The role of the thymus in the development of fatal lymphoreticular disease in the scurfy mouse was investigated. Neonatal thymectomy doubles the life span of scurfy mice, moderates the histologic lesions, and prevents anemia, despite the continued presence of high levels of serum IgG. Animals bred to be nude and scurfy (nu/nu;sf/Y) are viable, fertile, and free of scurfy lesions. Bone marrow from scurfy mice can reconstitute lethally irradiated, H-2-compatible animals but does not transmit scurfy disease. The authors conclude, from these data, that scurfy lesions are mediated by T lymphocytes that mature in an abnormal (sf) thymic environment.

• DOE Contract Number: AC05-84OR21400
• OSTI ID: 6096505
• Journal Information: Proceedings of the National Academy of Sciences of the United States of America; (United States), Vol. 88:13; ISSN 0027-8424
• Country of Publication: United States
• Language: English

Related Subjects

59 BASIC BIOLOGICAL SCIENCES
HEREDITARY DISEASES
ETIOLOGY
THYMOCYTES
CELL PROLIFERATION
ELECTROPHORESIS
HEMOGLOBIN
LYMPHATIC SYSTEM
MAN
MICE
RECESSIVE MUTATIONS
SKIN
THYMUS
X CHROMOSOME
ANIMAL CELLS
ANIMALS
BODY
CARBOXYLIC ACIDS
CHROMOSOMES
DISEASES
GLOBINS
HETEROCHROMOSOMES
HETEROCYCLIC ACIDS
HETEROCYCLIC COMPOUNDS
MAMMALS
MUTATIONS
ORGANIC ACIDS
ORGANIC COMPOUNDS
ORGANIC NITROGEN COMPOUNDS
ORGANS
PIGMENTS
PORPHYRINS
PRIMATES
PROTEINS
RODENTS
SOMATIC CELLS
VERTEBRATES
550400* - Genetics