Disruption of a new forkhead/winged-helix protein, scurfin, results in the fatal lymphoproliferative disorder of the scurfy mouse

Nature Genetics

Volumn 27, Issue 1, 2001, Pages 68-73

  Brunkow, Mary E ^a   Jeffery, Eric W ^a   Hjerrild, Kathryn A ^a   Paeper, Bryan ^a   Clark, Lisa B ^a   Yasayko, Sue Ann ^a   Wilkinson, J Erby ^b   Galas, David ^c   Ziegler, Steven F ^d   Ramsdell, Fred ^a  

^a Celltech Chiroscience Inc   (United States)

^b OAK RIDGE NATIONAL LABORATORY   (United States)

^c KECK GRADUATE INSTITUTE   (United States)

^d BENAROYA RESEARCH INSTITUTE   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

CD4 ANTIGEN; CD8 ANTIGEN; COMPLEMENTARY DNA; DNA BINDING PROTEIN; GENE PRODUCT; TRANSCRIPTION FACTOR FOXP3; UNCLASSIFIED DRUG;

ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; GENE LOCUS; GENE MAPPING; LYMPHOPROLIFERATIVE DISEASE; MOUSE; NONHUMAN; NUCLEOTIDE SEQUENCE; OPEN READING FRAME; PRIORITY JOURNAL; REVERSE TRANSCRIPTION POLYMERASE CHAIN REACTION; T LYMPHOCYTE; TRANSGENIC MOUSE; X CHROMOSOME LINKED DISORDER; X CHROMOSOME RECESSIVE DISORDER;

AMINO ACID MOTIFS; AMINO ACID SEQUENCE; ANIMALS; CLONING, MOLECULAR; CONSERVED SEQUENCE; DNA MUTATIONAL ANALYSIS; DNA-BINDING PROTEINS; FEMALE; FORKHEAD TRANSCRIPTION FACTORS; GENE EXPRESSION PROFILING; GENES, ESSENTIAL; GENES, RECESSIVE; GENETIC COMPLEMENTATION TEST; HUMANS; LYMPH NODES; LYMPHOCYTE COUNT; LYMPHOPROLIFERATIVE DISORDERS; MALE; MICE; MICE, MUTANT STRAINS; MICE, TRANSGENIC; MOLECULAR SEQUENCE DATA; MUTATION; PHENOTYPE; PHYSICAL CHROMOSOME MAPPING; PROTEIN STRUCTURE, TERTIARY; RNA, MESSENGER; SEQUENCE ALIGNMENT;

ANIMALIA; MUS MUSCULUS;

EID: 0035162560     PISSN: 10614036     EISSN: None     Source Type: Journal    
DOI: 10.1038/83784     Document Type: Article

References (31)

1. The scurfy mouse mutant has previously unrecognized hematological abnormalities and resembles Wiskott-Aldrich syndrome
2. Disease in the scurfy (sf) mouse is associated with overexpression of cytokine genes
3. CD4+CD8- T cells are the effector cells in disease pathogenesis in the scurfy (sf) mouse
4. Cellular and molecular characterization of the scurfy mouse mutant
5. Loss of CTLA-4 leads to massive lymphoproliferation and fatal multiorgan tissue destruction, revealing a critical negative regulatory role of CTLA-4
6. Lymphoproliferative disorders with early lethality in mice deficient in Ctla-4
7. Targeted disruption of the mouse transforming growth factor-β1 gene results in multifocal inflammatory disease
8. Transforming growth factor β 1 null mutation in mice causes excessive inflammatory response and early death
9. Transplantation of T cell-mediated, lymphoreticular disease from the scurfy (sf) mouse
10. The mouse scurfy (sf) mutation is tightly linked to Gata1 and Tfe3 on the proximal X chromosome
11. A transcript map of a 2-Mb BAC contig in the proximal portion of the mouse X chromosome and regional mapping of the scurfy mutation
12. Unified nomenclature for the winged-helix/forkhead transcription factors
13. A targeted mutation at the T-cell receptor α/δ locus impairs T-cell development and reveals the presence of the nearby antiapoptosis gene Dad1
14. Mouse X chromosome
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16. DNA-binding properties and secondary structural model of the hepatocyte nuclear factor 3/forkhead domain
17. Co-crystal structure of the HNF-3/forkhead DNA-recognition motif resembles histone H5
18. X-linked neonatal diabetes mellitus, enteropathy and endocrinopathy syndrome is the human equivalent of mouse scurfy
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20. Nuclear localization signals overlap DNA- or RNA-binding domains in nucleic acid-binding proteins
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