Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0043346 (xeroderma pigmentosum)
 2,924 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Cells of a human RSa cell line, with high sensitivity to UV killing and low capacity for DNA repair, when pretreated with 1-100 units/ml of human interferon (HuIFN) preparations for more than 12 h before irradiation, acquired an enhancement of UV-induced DNA-repair replication synthesis in association with recovery from inhibition of total cellular DNA synthesis and UV survival. Prompt and transient induction of plasminogen activator activities was also found within 5 min after UV irradiation in the cells pretreated with HuIFN but not in the cells non-pretreated with HuIFN. The enhancement and induction effects of HuIFN were observed, irrespective of the kind of HuIFN preparation used (alpha, beta or gamma, and natural or recombinant) and in other UV-sensitive fibroblast cells which were derived from Cockayne syndrome and xeroderma pigmentosum fibroblasts (XP1KY). However, all of the enhancement of DNA-repair synthesis and the induction of plasminogen activator activities by HuIFN was suppressed by treatment with cycloheximide immediately after UV irradiation.
 ...
PMID:Effects of human interferon on cellular response to UV in UV-sensitive human cell strains. 245 Nov 28

DNA repair synthesis and strand break DNA repair induced by 4-nitroquinoline-1-oxide and UV-irradiation in Xeroderma pigmentosum lymphocytes and fibroblasts pretreated by leucocyte interferons were studied. Stimulation of DNA repair synthesis in interferon-pretreated Xeroderma pigmentosum cells, defective in incision, was detected. No such effect was noted for strand break DNA repair. Hence, antimutagenic activity of interferons in human cells is connected with their modificating effect on DNA repair.
 ...
PMID:[Mechanisms of impairment of DNA repair in human cells. Interferons stimulated DNA repair in xeroderma pigmentosum cells]. 251 59

Effects of native and recombinant interferons in normal and xeroderma pigmentosum (XP) human fibroblasts were studied. The criteria to evaluate the effects of interferons were: inhibition of replicative DNA synthesis, modification of replicative and unscheduled DNA synthesis in UV-irradiated cells. It was demonstrated that the level of inhibition of replicative DNA synthesis with interferons was dependent on proliferative status of cell cultures. The level of 3H-thymidine incorporation after stimulation of cell proliferation was increased in UV-irradiated normal fibroblasts pretreated with interferons. Interferon effect was not observed in XP cells. In cell cultures with low level of proliferation treatment with interferon resulted in inhibition of DNA replication and pronounced increase in the unscheduled DNA synthesis. In quiescent cells the effect of interferon on unscheduled DNA synthesis in UV-irradiated cells was not pronounced. In XP cells UV-irradiation induced low unscheduled DNA synthesis which was not modified by interferon.
 ...
PMID:[Mechanisms of impairment of DNA repair in human cells. Effects of native and recombinant interferons in UV-irradiated human fibroblasts]. 262 3

Effects of human interferon (HuIFN)-alpha on UV mutagenicity were examined in a human cell strain, RSa, and xeroderma pigmentosum (XP)-derived fibroblasts (XP1KY). The frequency of ouabain-resistance mutation in UV-irradiated RSa cells was unusually high (Suzuki et al., 1985), but that in cells pretreated with HuIFN-alpha before irradiation was reduced. 6-Thioguanine-resistance mutation was also depressed in XP1KY cells treated with HuIFN-alpha before irradiation. However, the depression of UV mutagenicity by HuIFN-alpha was lessened by treatment with cycloheximide immediately after UV irradiation. The relationship between HuIFN-depressed UV mutagenicity and HuIFN-affected DNA-repair and repair-related functions is discussed.
 ...
PMID:Suppression of UV mutagenicity by human interferon. 318 89

Correlation between susceptibility to the anticellular effect of human interferon (HuIFN) and ultraviolet (uv) lethality was examined in a set of isogeneous human cell lines (RSa and IFr cells), a human endometrial cancer cell line (HEC-1 cells), and a Xeroderma pigmentosum-derived fibroblast cell line ( CRL1200 cells). IFr cells, previously established as a HuIFN-alpha-resistant subline by exposing HuIFN-alpha- and uv-sensitive RSa cells to HuIFN-alpha preparations, appeared more refractory to uv than did the parent RSa cells in the cell proliferation and colony-formation studies. The extent of recovery from uv-inhibited total cellular DNA synthesis and uv-induced DNA-repair synthesis was enhanced to a greater extent in IFr cells than in RSa cells. The preexposure of RSa cells to HuIFN-alpha enhanced uv-induced DNA-repair replication activities. HEC-1 cells, which are reportedly totally refractory to HuIFN actions, appeared most resistant to uv, in all the tests. The uv-sensitive CRL1200 cells appeared highly susceptible to HuIFN-beta, in both cell proliferation and DNA-synthesis inhibition tests. These results support and extend our previous finding (N. Suzuki, J. Nishimaki , and T. Kuwata (1982). Mutat . Res. 106, 357-376) that susceptibility to the anticellular effect of HuIFN closely relates with uv lethality.
 ...
PMID:Cross-sensitivity between interferon and uv in human cell strains: IFr, HEC-1, and CRL1200. 673 Mar 35

Natural leucocyte interferon (IF) upon injection to two patients affected with Xeroderma pigmentosum for three weeks stimulated the inhibited DNA replication and Host reactivation at the level of healthy donors. Tigasol--the drug used traditionally for the treatment of diseases caused by Xeroderma pigmentosum proved less effective than IF.
 ...
PMID:[Interferon as a stimulator of DNA repair in patients with xeroderma pigmentosum]. 750 9

To study the relationship between the transient elevation of protease activity and hypomutability observed in hypermutable human RSa cells pretreated with human interferon (HuIFN)-alpha and then irradiated with far-ultraviolet light (UV), protease inhibitors capable of specifically inhibiting the activity were investigated. Of ten inhibitors tested, antipain showed the greatest inhibitory effect. Antipain also prevented the suppression of UV-mutagenicity by HuIFN-alpha in RSa and xeroderma pigmentosum-derived fibroblast cells, as shown by culturing cells in medium containing antipain immediately after UV exposure and evaluating the generation of clones resistant to ouabain- or 6-thioguanine-mediated cytotoxicity. Thus, an antipain-sensitive protease may be involved in the hypomutability induced by HuIFN-alpha.
 ...
PMID:Involvement of antipain-sensitive protease activity in suppression of UV-mutagenicity by human interferon-alpha. 752 35

Xeroderma pigmentosum (XP) is a rare autosomal recessive disorder with sun sensitivity, markedly increased skin cancer susceptibility, and defective DNA repair without consistently identified symptoms of immune deficiency. We examined natural killer (NK) cell activity and interferon production in peripheral blood lymphocytes (PBL) of eight XP patients who had multiple primary skin cancers. The XP patients had normal numbers of T cells and NK cells, as well as normal lymphokine-activated killer cell activity and normal tumor necrosis factor-alpha production. Unstimulated NK cell function was 40% of normal controls in five XP patients, but was normal in three other XP patients. However, PBL from all the XP patients tested showed no enhancement of NK activity by the interferon inducer, polyinosinic acid:polycytidilic acid (polyIC) but enhancement by interferon-alpha was normal, suggesting an impairment in interferon production. Parallel studies in non-XP skin cancer patients revealed that both unstimulated and polyIC-enhanced NK activity were normal. Further investigation using PBL from XP patients revealed that the production of interferon-gamma after stimulation with interferon inducers (polyIC, interleukin 2, or K562 tumor cells) was 13-43% of normals. These data indicate that XP lymphocytes have a defect in production of interferons and suggest that defective interferon production, as well as DNA repair defects, may play an important role in the susceptibility of XP patients to skin cancer.
 ...
PMID:Impaired interferon production and natural killer cell activation in patients with the skin cancer-prone disorder, xeroderma pigmentosum. 769 Jul 72

Using PCR-differential display, we have searched for genes expressed specially in human interferon (HuIFn)-beta-treated Cockayne syndrome (CS) fibroblast cells. Eighteen expressed genes induced by HuIFN-beta were identified, the sequences of seven of which were highly homologous to previously cloned sequences. The cDNAs of six of these seven clones were similar to expression tagged sequences from unknown genes in databases and the remaining one was identical to the cDNA of the xeroderma pigmentosum XPG gene. These results, together with our previous finding of increased resistance to ultraviolet (UV) cell-killing of CS cells pretreated with HuIFN-beta prior to UV irradiation suggest that XPG might be one of the genes possibly involved in the HuIFN-beta-induced UV-resistance.
 ...
PMID:Enhancement of XPG mRNA expression by human interferon-beta in Cockayne syndrome cells. 967 65

Basal cell carcinoma is the most frequent of all cancers. Its incidence has risen those last decades because of the increase in sun exposure habits. People with light skin complexion are particularly at risk. Ionizing radiations, arsenicism, various genodermatoses (Gorlin syndrome, xeroderma pigmentosum, nevus sebaceous) are other pre-disposing factors. Basal cell carcinoma usually present as small lesion with a pearly border and telangiectasias. Other clinical types must also be recognized such as the nodular, the infiltrative, the superficial and the pigmented forms. They are usually located on the head and trunk. The prognosis of basal cell carcinoma is usually good since they can be cured by surgical excision and because they usually do not metastasize. However, large, multiple or recurrent basal cell carcinomas can be difficult to treat. In these cases, cryosurgery, radiotherapy or intralesional interferon-alfa may be needed.
 ...
PMID:[Basal cell carcinoma]. 1033 93


1 2 Next >>