AI Article Synopsis
- XP groups A and G xeroderma pigmentosum (XP) cells show temporary recovery of excision repair deficiencies after HeLa poly(A)+RNA microinjection, while groups D and F do not.
- Microinjection of total poly(A)+RNA from HeLa cells enhances unscheduled DNA synthesis (UDS) particularly in XP-A cells but also in XP-E and XP-G cells when using a more concentrated RNA fraction.
- The study provides estimates of mRNA sizes for XP-E and XP-G proteins, aiding future molecular cloning of DNA repair genes related to these groups.
Article Abstract
Excision repair deficiencies in groups A and G xeroderma pigmentosum (XP) cells are transiently complemented after microinjection of HeLa poly(A)+RNA, but those in groups D and F are not complemented (Legerski et al., 1984). We tested XP cells belonging to the seven complementation groups, A-G, and Cockayne's syndrome (CS) cells belonging to the two complementation groups, A and B, for transient correction by microinjection of total poly(A)+RNA from HeLa cells. Among the XP cells, unscheduled DNA synthesis (UDS) was increased only in XP-A cells by microinjection of total poly(A)+RNA. However, UDS was increased in XP-E and XP-G cells as well as in XP-A cells by microinjection of concentrated poly(A)+RNA fractionated on a 5-25% sucrose density gradient containing methylmercuric hydroxide. The sizes of XP-E and XP-G mRNA were estimated to be 1.5-2.7 kb and 2.0-3.8 kb, respectively, by comparison to internal marker RNAs including 18S rRNA, 28S rRNA, HPRT mRNA and XPAC mRNA. RNA synthesis recovery after UV exposure in CS cells was not increased by microinjection of either total poly(A)+RNA or fractionated RNA. These results provide estimates of the sizes of XP-E and XP-G proteins and will facilitate molecular cloning of DNA repair genes, especially of XP-E and XP-G genes.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1016/0921-8777(94)90056-6 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Novel mutation identified in the DDB2 gene in patients with xeroderma pigmentosum group-E.
Int J Dermatol
August 2020
Duzce University Medical School, Duzce, Turkey.
Background: Xeroderma pigmentosum (XP) is a rare photosensitive syndrome, which is divided into eight complementation groups (XP-A to XP-G and XPV) and characterized by skin cancers diagnosed at early age. A family of seven members (age range between 5 and 47 years) with carriers of the novel nonsense mutation that causes XP-E type were included in the current study.
Methods: DNA was isolated from peripheral blood samples of the proband, and cancer predisposition genes were sequenced with next-generation sequencing.
Deep phenotyping of 89 xeroderma pigmentosum patients reveals unexpected heterogeneity dependent on the precise molecular defect.
Proc Natl Acad Sci U S A
March 2016
Genome Damage and Stability Centre, University of Sussex, Falmer, Brighton BN1 9RQ, United Kingdom;
Xeroderma pigmentosum (XP) is a rare DNA repair disorder characterized by increased susceptibility to UV radiation (UVR)-induced skin pigmentation, skin cancers, ocular surface disease, and, in some patients, sunburn and neurological degeneration. Genetically, it is assigned to eight complementation groups (XP-A to -G and variant). For the last 5 y, the UK national multidisciplinary XP service has provided follow-up for 89 XP patients, representing most of the XP patients in the United Kingdom.
View Article and Find Full Text PDFPatients with xeroderma pigmentosum complementation groups C, E and V do not have abnormal sunburn reactions.
Br J Dermatol
December 2013
UK National Xeroderma Pigmentosum Service, Department of Photodermatology, St John's Institute of Dermatology, Guy's and St Thomas' NHS Trust, London, U.K.
Background: Xeroderma pigmentosum (XP) is a rare autosomal recessive disorder of DNA repair. It is divided into eight complementation groups: XP-A to XP-G (classical XP) and XP variant (XP-V). Severe and prolonged sunburn reactions on minimal sun exposure have been considered a cardinal feature of classical XP.
View Article and Find Full Text PDFGene expression profiling of xeroderma pigmentosum.
Hered Cancer Clin Pract
May 2006
Hunter Medical Research Institute, John Hunter Hospital, Lookout Rd, New Lambton Heights, NSW, 2305, Australia.
Xeroderma pigmentosum (XP) is a rare recessive disorder that is characterized by extreme sensitivity to UV light. UV light exposure results in the formation of DNA damage such as cyclobutane dimers and (6-4) photoproducts. Nucleotide excision repair (NER) orchestrates the removal of cyclobutane dimers and (6-4) photoproducts as well as some forms of bulky chemical DNA adducts.
View Article and Find Full Text PDFNucleotide excision repair-deficient human cells in culture exhibit decreased survival after 2-chlorodeoxyadenosine treatment.
Anticancer Res
September 2003
Department of Pharmacology, University of Health Sciences, 1750 Independence Avenue, Kansas City, Missouri 64106, USA.
2-Chloro-2'-deoxyadenosine (CldAdo, cladribine) is a clinically important nucleoside analog for adult and pediatric leukemias. We previously described an activity in HeLa cell nuclear extracts that specifically recognized CldAMP-substituted oligomers. The factor was present in extracts prepared from repair-deficient xeroderma pigmentosum (XP) complementation group A cells, but not from group E--which are defective in damaged DNA-binding (DDB) protein--suggesting a possible repair process for incorporated analogs.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!