TRAF6-deficient mice display hypohidrotic ectodermal dysplasia
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چکیده
منابع مشابه
TRAF6-deficient mice display hypohidrotic ectodermal dysplasia.
Tumor necrosis factor receptor (TNFR)-associated factor 6 (TRAF6) is an adapter protein that links signals from members of the TNFR superfamily and Toll/IL-1 receptor family to activation of transcription factors NFkappaB and AP-1. Analysis of TRAF6-deficient mice revealed that TRAF6 is essential for normal bone formation and establishment of immune and inflammatory systems. Here we report that...
متن کاملHypohidrotic ectodermal dysplasia.
X linked hypohidrotic ectodermal dysplasia was studied in the dentition of both affected males and carrier females. Hypodontia was more severe in males than females and there were differences in the pattern of tooth absence between the sexes. Abnormal crown form, with the maximum diameter of the teeth being apically displaced, was noted particulady in the anterior teeth. Taurodontism was common...
متن کاملAtopic diathesis in hypohidrotic/anhidrotic ectodermal dysplasia.
Recently, patients with hypohidrotic/anhidrotic ectodermal dysplasia (H/AED) have been reported to have a higher prevalence of symptoms suggestive of atopic disorders than the general population. To better understand atopic diathesis in H/AED, 6 cases of clinically or genetically diagnosed H/AED were examined. The following criteria were evaluated with patient consent: sweating, blood test resu...
متن کاملEarly recognition of hypohidrotic ectodermal dysplasia
Hypohidrotic ectodermal dysplasia (HED) is characterized by severe hypohidrosis, hypoplasia of sweat, sebaceous, submucous, meibomian and mammary glands, hypotrichosis, and oligodontia. In early childhood, HED is a life-threatening disorder based on the risks for hyperthermia and pneumonia. Awareness of the hazards related to this rare genetic disease is most helpful in preventing avoidable cal...
متن کاملHypohidrotic Ectodermal Dysplasia: Prosthetic and Endodontic Management
Individuals affected by ectodermal dysplasia syndromes have abnormalities of the glands, tooth buds, hair follicles, and nail development. Oral finding in ectodermal dysplasia patient are significant and can include multiple abnormalities of the dentition such as anodontia, hy-podontia or malformed and widely spaced peg like teeth, loss of occlusal vertical dimension, protuberant lips and lack ...
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ژورنال
عنوان ژورنال: Proceedings of the National Academy of Sciences
سال: 2002
ISSN: 0027-8424,1091-6490
DOI: 10.1073/pnas.132636999