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. |