Lipoid Proteinosis of Urbach and Wiethe
"Lipoid Proteinosis of Urbach and Wiethe" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus,
MeSH (Medical Subject Headings). Descriptors are arranged in a hierarchical structure,
which enables searching at various levels of specificity.
An autosomal recessive disorder characterized by glassy degenerative thickening (hyalinosis) of SKIN; MUCOSA; and certain VISCERA. This disorder is caused by mutation in the extracellular matrix protein 1 gene (ECM1). Clinical features include hoarseness and skin eruption due to widespread deposition of HYALIN.
| Descriptor ID |
D008065
|
| MeSH Number(s) |
C08.618.490.500 C16.320.850.595
|
| Concept/Terms |
Lipoid Proteinosis of Urbach and Wiethe- Lipoid Proteinosis of Urbach and Wiethe
- Lipoidproteinosis
- Urbach-Wiethe Syndrome
- Urbach-Wiethe Disease
- Urbach Wiethe Disease
- Urbach-Wiethe Lipoid Proteinosis
- Lipoid Proteinosis, Urbach-Wiethe
- Urbach Wiethe Lipoid Proteinosis
- Hyalinosis Cutis et Mucosae
- Lipoproteinosis
|
Below are MeSH descriptors whose meaning is more general than "Lipoid Proteinosis of Urbach and Wiethe".
Below are MeSH descriptors whose meaning is more specific than "Lipoid Proteinosis of Urbach and Wiethe".
This graph shows the total number of publications written about "Lipoid Proteinosis of Urbach and Wiethe" by people in this website by year, and whether "Lipoid Proteinosis of Urbach and Wiethe" was a major or minor topic of these publications.
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| Year | Major Topic | Minor Topic | Total |
|---|
| 2002 | 1 | 0 | 1 |
| 2003 | 1 | 0 | 1 |
| 2004 | 1 | 0 | 1 |
| 2006 | 0 | 1 | 1 |
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Below are the most recent publications written about "Lipoid Proteinosis of Urbach and Wiethe" by people in Profiles.
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Fujimoto N, Terlizzi J, Aho S, Brittingham R, Fertala A, Oyama N, McGrath JA, Uitto J. Extracellular matrix protein 1 inhibits the activity of matrix metalloproteinase 9 through high-affinity protein/protein interactions. Exp Dermatol. 2006 Apr; 15(4):300-7.
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Chan I, South AP, McGrath JA, Oyama N, Bhogal BS, Black MM, Hamada T. Rapid diagnosis of lipoid proteinosis using an anti-extracellular matrix protein 1 (ECM1) antibody. J Dermatol Sci. 2004 Aug; 35(2):151-3.
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Hamada T, Wessagowit V, South AP, Ashton GH, Chan I, Oyama N, Siriwattana A, Jewhasuchin P, Charuwichitratana S, Thappa DM, Jeevankumar B, Lenane P, Krafchik B, Kulthanan K, Shimizu H, Kaya TI, Erdal ME, Paradisi M, Paller AS, Seishima M, Hashimoto T, McGrath JA. Extracellular matrix protein 1 gene (ECM1) mutations in lipoid proteinosis and genotype-phenotype correlation. J Invest Dermatol. 2003 Mar; 120(3):345-50.
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Hamada T, McLean WH, Ramsay M, Ashton GH, Nanda A, Jenkins T, Edelstein I, South AP, Bleck O, Wessagowit V, Mallipeddi R, Orchard GE, Wan H, Dopping-Hepenstal PJ, Mellerio JE, Whittock NV, Munro CS, van Steensel MA, Steijlen PM, Ni J, Zhang L, Hashimoto T, Eady RA, McGrath JA. Lipoid proteinosis maps to 1q21 and is caused by mutations in the extracellular matrix protein 1 gene (ECM1). Hum Mol Genet. 2002 Apr 1; 11(7):833-40.