The Experts below are selected from a list of 258 Experts worldwide ranked by ideXlab platform
Shimon Ullman - One of the best experts on this subject based on the ideXlab platform.
-
Xeroderma pigmentosum lesions related to ultraviolet transmittance by clothes
Journal of the American Academy of Dermatology, 1991Co-Authors: Niels Bech-thomsen, Hans Christian Wulf, Shimon UllmanAbstract:Xeroderma pigmentosum is associated with severe actinic degeneration of the skin. Our patient with Xeroderma pigmentosum showed increasing actinic damage in some areas covered by clothes. We therefore performed a complete evaluation of the patienťs exposure to ultraviolet radiation. This included transmission measurements of the patient's clothes, glasses, and car windows. The transmission of UVB (280 to 320 nm) by the clothes varied from none to 17.8%. The transmitted UVB radiation was proportional to the clinical manifestations of Xeroderma pigmentosum. The patienťs wardrobe was changed, and UVA-blocking film was applied to the windows of the car and house. A decline in manifestations was seen after 18 months of improved ultraviolet protection. © 1991, American Academy of Dermatology, Inc. All rights reserved.
Niels Bech-thomsen - One of the best experts on this subject based on the ideXlab platform.
-
Xeroderma pigmentosum lesions related to ultraviolet transmittance by clothes
Journal of The American Academy of Dermatology, 1991Co-Authors: Niels Bech-thomsen, Hans Christian Wulf, Susanne UllmanAbstract:Xeroderma pigmentosum is associated with severe actinic degeneration of the skin. Our patient with Xeroderma pigmentosum showed increasing actinic damage in some areas covered by clothes. We therefore performed a complete evaluation of the patienťs exposure to ultraviolet radiation. This included transmission measurements of the patient's clothes, glasses, and car windows. The transmission of UVB (280 to 320 nm) by the clothes varied from none to 17.8%. The transmitted UVB radiation was proportional to the clinical manifestations of Xeroderma pigmentosum. The patienťs wardrobe was changed, and UVA-blocking film was applied to the windows of the car and house. A decline in manifestations was seen after 18 months of improved ultraviolet protection.
-
Xeroderma pigmentosum lesions related to ultraviolet transmittance by clothes
Journal of the American Academy of Dermatology, 1991Co-Authors: Niels Bech-thomsen, Hans Christian Wulf, Shimon UllmanAbstract:Xeroderma pigmentosum is associated with severe actinic degeneration of the skin. Our patient with Xeroderma pigmentosum showed increasing actinic damage in some areas covered by clothes. We therefore performed a complete evaluation of the patienťs exposure to ultraviolet radiation. This included transmission measurements of the patient's clothes, glasses, and car windows. The transmission of UVB (280 to 320 nm) by the clothes varied from none to 17.8%. The transmitted UVB radiation was proportional to the clinical manifestations of Xeroderma pigmentosum. The patienťs wardrobe was changed, and UVA-blocking film was applied to the windows of the car and house. A decline in manifestations was seen after 18 months of improved ultraviolet protection. © 1991, American Academy of Dermatology, Inc. All rights reserved.
Alain Sarasin - One of the best experts on this subject based on the ideXlab platform.
-
XPE (Xeroderma pigmentosum, complementation group E)
Atlas of genetics and cytogenetics in oncology and haematology, 2011Co-Authors: Anne Stary, Alain SarasinAbstract:Review on XPE (Xeroderma pigmentosum, complementation group E), with data on DNA, on the protein encoded, and where the gene is implicated.
-
ERCC5 (Xeroderma pigmentosum, complementation group G)
Atlas of genetics and cytogenetics in oncology and haematology, 2011Co-Authors: Anne Stary, Alain SarasinAbstract:Review on ERCC5(Xeroderma pigmentosum, complementation group G), with data on DNA, on the protein encoded, and where the gene is implicated.
-
XPC (Xeroderma pigmentosum, complementation group C)
Atlas of genetics and cytogenetics in oncology and haematology, 2011Co-Authors: Anne Stary, Alain SarasinAbstract:Review on XPC (Xeroderma pigmentosum, complementation group C), with data on DNA, on the protein encoded, and where the gene is implicated.
-
XPA (Xeroderma pigmentosum, complementation group A)
Atlas of genetics and cytogenetics in oncology and haematology, 2011Co-Authors: Anne Stary, Alain SarasinAbstract:Review on XPA (Xeroderma pigmentosum, complementation group A), with data on DNA, on the protein encoded, and where the gene is implicated.
-
ERCC4 (Xeroderma pigmentosum, complementation group F)
Atlas of genetics and cytogenetics in oncology and haematology, 2011Co-Authors: Anne Stary, Alain SarasinAbstract:Review on ERCC4 (Xeroderma pigmentosum, complementation group F), with data on DNA, on the protein encoded, and where the gene is implicated.
Hans Christian Wulf - One of the best experts on this subject based on the ideXlab platform.
-
Xeroderma pigmentosum lesions related to ultraviolet transmittance by clothes
Journal of The American Academy of Dermatology, 1991Co-Authors: Niels Bech-thomsen, Hans Christian Wulf, Susanne UllmanAbstract:Xeroderma pigmentosum is associated with severe actinic degeneration of the skin. Our patient with Xeroderma pigmentosum showed increasing actinic damage in some areas covered by clothes. We therefore performed a complete evaluation of the patienťs exposure to ultraviolet radiation. This included transmission measurements of the patient's clothes, glasses, and car windows. The transmission of UVB (280 to 320 nm) by the clothes varied from none to 17.8%. The transmitted UVB radiation was proportional to the clinical manifestations of Xeroderma pigmentosum. The patienťs wardrobe was changed, and UVA-blocking film was applied to the windows of the car and house. A decline in manifestations was seen after 18 months of improved ultraviolet protection.
-
Xeroderma pigmentosum lesions related to ultraviolet transmittance by clothes
Journal of the American Academy of Dermatology, 1991Co-Authors: Niels Bech-thomsen, Hans Christian Wulf, Shimon UllmanAbstract:Xeroderma pigmentosum is associated with severe actinic degeneration of the skin. Our patient with Xeroderma pigmentosum showed increasing actinic damage in some areas covered by clothes. We therefore performed a complete evaluation of the patienťs exposure to ultraviolet radiation. This included transmission measurements of the patient's clothes, glasses, and car windows. The transmission of UVB (280 to 320 nm) by the clothes varied from none to 17.8%. The transmitted UVB radiation was proportional to the clinical manifestations of Xeroderma pigmentosum. The patienťs wardrobe was changed, and UVA-blocking film was applied to the windows of the car and house. A decline in manifestations was seen after 18 months of improved ultraviolet protection. © 1991, American Academy of Dermatology, Inc. All rights reserved.
Sergij Goerdt - One of the best experts on this subject based on the ideXlab platform.
-
molecular genetics of Xeroderma pigmentosum variant
Experimental Dermatology, 2003Co-Authors: Alexei Gratchev, Pamela Strein, Jochen Utikal, Sergij GoerdtAbstract:Abstract: Xeroderma pigmentosum (XP) is an autosomal recessive disease characterized by sun sensitivity, early onset of freckling and subsequent neoplastic changes on sun-exposed skin. Skin abnormalities result from an inability to repair UV-damaged DNA because of defects in the nucleotide excision repair (NER) machinery. Xeroderma pigmentosum is genetically heterogeneous and is classified into seven complementation groups (XPA-XPG) that correspond to genetic alterations in one of seven genes involved in NER. The variant type of XP (XPV), first described in 1970 by Ernst G. Jung as ‘pigmented xerodermoid’, is caused by defects in the post replication repair machinery while NER is not impaired. Identification of the XPV gene was only achieved in 1999 by biochemical purification and sequencing of a protein from HeLa cell extracts complementing the PRR defect in XPV cells. The XPV protein, polymerase (pol)η, represents a novel member of the Y family of bypass DNA polymerases that facilitate DNA translesion synthesis. The major function of polη is to allow DNA translesion synthesis of UV-induced TT-dimers in an error-free manner; it also possesses the capability to bypass other DNA lesions in an error-prone manner. Xeroderma pigmentosum V is caused by molecular alterations in the POLH gene, located on chromosome 6p21.1–6p12. Affected individuals are homozygous or compound heterozygous for a spectrum of genetic lesions, including nonsense mutations, deletions or insertions, confirming the autosomal recessive nature of the condition. Identification of POLH as the XPV gene provides an important instrument for improving molecular diagnostics in XPV families.
