PubMed:20854438 JSONTXT 30 Projects

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Id Subject Object Predicate Lexical cue
T1 0-74 Sentence denotes SLURP1 mutation-impaired T-cell activation in a family with mal de Meleda.
T2 75-86 Sentence denotes BACKGROUND:
T3 87-326 Sentence denotes Mal de Meleda (MDM) is palmoplantar erythrokeratoderma with an autosomal recessive inheritance and is caused by a mutation in the gene encoding SLURP-1 (lymphocyte antigen 6/urokinase-type plasminogen activator receptor related protein-1).
T4 327-447 Sentence denotes SLURP-1 is an allosteric agonist to the nicotinic acetylcholine receptor (nAchR) and it regulates epidermal homeostasis.
T5 448-560 Sentence denotes In addition, murine studies have shown that nAchR signalling is important for the regulation of T-cell function.
T6 561-809 Sentence denotes Among the family members, patients with the homozygous SLURP1 (previously known as ARS component B) mutation are prone to melanoma and viral infection, which might link to defective T-cell function as well as a derangement of epidermal homeostasis.
T7 810-821 Sentence denotes OBJECTIVES:
T8 822-935 Sentence denotes To investigate the association of the SLURP1 gene mutation with T-cell activation in a Taiwanese family with MDM.
T9 936-992 Sentence denotes To test that SLURP-1 is essential for T-cell activation.
T10 993-1001 Sentence denotes METHODS:
T11 1002-1262 Sentence denotes Human peripheral blood mononuclear cells (PBMCs) were isolated from a Taiwanese MDM family bearing the G to A substitution in nucleotide 256 in the SLURP1 gene, corresponding to a glycine to arginine substitution at amino acid 86 (G86R) in the SLURP-1 protein.
T12 1263-1435 Sentence denotes PBMCs from homozygotes and wild-type controls were stimulated with anti-CD3/anti-CD28 antibodies and the level of T-cell activation was determined by the stimulation index.
T13 1436-1542 Sentence denotes RESULTS: PBMCs with the heterozygous and homozygous SLURP-1 G86R mutation had defective T-cell activation.
T14 1543-1628 Sentence denotes This was restored by the addition of 0·5 μg mL(-1) recombinant human SLURP-1 protein.
T15 1629-1641 Sentence denotes CONCLUSIONS:
T16 1642-1741 Sentence denotes Patients with MDM with the homozygous SLURP-1 G86R mutation may have an impaired T-cell activation.
T17 1742-1818 Sentence denotes The presence of wild-type SLURP-1 is essential for normal T-cell activation.