| pubmed-article:12084440 | rdf:type | pubmed:Citation | lld:pubmed |
| pubmed-article:12084440 | lifeskim:mentions | umls-concept:C0332307 | lld:lifeskim |
| pubmed-article:12084440 | lifeskim:mentions | umls-concept:C0229613 | lld:lifeskim |
| pubmed-article:12084440 | lifeskim:mentions | umls-concept:C0162638 | lld:lifeskim |
| pubmed-article:12084440 | lifeskim:mentions | umls-concept:C0028064 | lld:lifeskim |
| pubmed-article:12084440 | lifeskim:mentions | umls-concept:C0086982 | lld:lifeskim |
| pubmed-article:12084440 | lifeskim:mentions | umls-concept:C0376315 | lld:lifeskim |
| pubmed-article:12084440 | pubmed:issue | 1-2 | lld:pubmed |
| pubmed-article:12084440 | pubmed:dateCreated | 2002-6-26 | lld:pubmed |
| pubmed-article:12084440 | pubmed:abstractText | Deficient activity of human lysosomal hydrolase, acid sphingomyelinase (ASM), results in the neuronopathic (type A) and non-neuronopathic (type B) forms of Niemann-Pick disease (NPD). A deficiency of ASM is known to deprive lymphoblasts of their response to apoptotic induction by X-ray irradiation. To elucidate the genetic heterogeneity of apoptotic induction in NPD cells, we investigated radiation-induced apoptosis of lymphoblasts in patients with type A (genotype: IVS3-2A-G/IVS3-2A-G) and type B (genotype: S436R/S436R) NPD. Epstein-Barr virus (EBV)-transformed lymphoblasts established from a patient with type A NPD, a patient with type B NPD and a normal control were irradiated with 20 Gy and incubated for 24 h. The cells were harvested and the morphological features of apoptosis were observed with DNA-specific fluorochrome bis-benzimide. Exposure of lymphoblasts to 20 Gy of radiation resulted in 25-30% apoptosis of total cells in normal lymphoblasts, 8-9% apoptosis in type A NPD and 20-27% apoptosis in type B NPD. The radiation-induced apoptotic response in the lymphoblasts of type A NPD was significantly different from that of the normal lymphoblasts (P<0.0005). On the other hand, the radiation-induced apoptotic response in type B NPD was not markedly different from that in normal lymphoblasts (P=0.624). In the patient with type B NPD, the signaling pathway for radiation-induced apoptosis was preserved in lymphoblasts, which suggests that the extent of cell signaling system disturbance due to ASM deficiency may be related to the phenotypes in types A and B NPD. | lld:pubmed |
| pubmed-article:12084440 | pubmed:language | eng | lld:pubmed |
| pubmed-article:12084440 | pubmed:journal | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:12084440 | pubmed:citationSubset | IM | lld:pubmed |
| pubmed-article:12084440 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:12084440 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:12084440 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:12084440 | pubmed:status | MEDLINE | lld:pubmed |
| pubmed-article:12084440 | pubmed:month | Jul | lld:pubmed |
| pubmed-article:12084440 | pubmed:issn | 0022-510X | lld:pubmed |
| pubmed-article:12084440 | pubmed:author | pubmed-author:TakadaGoroG | lld:pubmed |
| pubmed-article:12084440 | pubmed:author | pubmed-author:IshidaAkiraA | lld:pubmed |
| pubmed-article:12084440 | pubmed:author | pubmed-author:TakahashiTsut... | lld:pubmed |
| pubmed-article:12084440 | pubmed:author | pubmed-author:MikamiTamakiT | lld:pubmed |
| pubmed-article:12084440 | pubmed:author | pubmed-author:MinamiyaYoshi... | lld:pubmed |
| pubmed-article:12084440 | pubmed:author | pubmed-author:IdaHiroyukiH | lld:pubmed |
| pubmed-article:12084440 | pubmed:issnType | Print | lld:pubmed |
| pubmed-article:12084440 | pubmed:day | 15 | lld:pubmed |
| pubmed-article:12084440 | pubmed:volume | 199 | lld:pubmed |
| pubmed-article:12084440 | pubmed:owner | NLM | lld:pubmed |
| pubmed-article:12084440 | pubmed:authorsComplete | Y | lld:pubmed |
| pubmed-article:12084440 | pubmed:pagination | 39-43 | lld:pubmed |
| pubmed-article:12084440 | pubmed:dateRevised | 2004-11-17 | lld:pubmed |
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| pubmed-article:12084440 | pubmed:meshHeading | pubmed-meshheading:12084440... | lld:pubmed |
| pubmed-article:12084440 | pubmed:year | 2002 | lld:pubmed |
| pubmed-article:12084440 | pubmed:articleTitle | Signaling pathway for radiation-induced apoptosis in the lymphoblasts from neuronopathic (type A) and non-neuronopathic (type B) forms of Niemann-Pick disease. | lld:pubmed |
| pubmed-article:12084440 | pubmed:affiliation | Department of Pediatrics, Akita University School of Medicine, Akita 010-8543, Japan. | lld:pubmed |
| pubmed-article:12084440 | pubmed:publicationType | Journal Article | lld:pubmed |
| http://linkedlifedata.com/r... | pubmed:referesTo | pubmed-article:12084440 | lld:pubmed |
