The scurfy mouse mutant has previously unrecognized hematological abnormalities and resembles Wiskott-Aldrich syndrome.

• DOI: 10.1073/pnas.87.7.2433
• Publication Date: 2006-05-31T11:35:54Z
• Authors: M F Lyon; J Peters; P H Glenister; S Ball; E Wright
• Keywords: Wiskott–Aldrich syndrome; Immunodeficiency Syndrome; Wiskott–Aldrich syndrome protein

The X chromosome-linked scurfy (sf) mutant of the mouse is recognized by scaliness skin from which name derived and results in death affected males at about 3-4 weeks age. Consideration known man-mouse homologies chromosome prompted hematological studies, have shown that blood highly abnormal. platelet erythrocyte counts are both reduced become progressively lower relative to normal as disease progresses. There gastrointestinal bleeding, most animals appear die severe anemia. By contrast, leukocyte count consistently raised. Some showed signs infection but it not yet clear whether there immunodeficiency. Other features include scaly apparently lateral growth skin, conjunctivitis, diarrhea some animals. resembles Wiskott-Aldrich syndrome man, characterized thrombocytopenia, eczema, diarrhea, loci human genes lie homologous segments chromosome, although somewhat different positions other gene loci. Scurfy differs hypogonadal.