Fibrillar protein deposits with tubular substructure in a systemic disease beginning as cutis laxa.

Fibrillar protein deposits with tubular substructure in a systemic disease beginning as cutis laxa. Research Abstract Details 

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Fibrillar protein deposits with tubular substructure in a systemic disease beginning as cutis laxa. Abstract Text:

BACKGROUND--The homogeneous material found in the skin is commonly identified as amyloid. We describe a previously unknown disease that is caused by proteinaceous deposits and that does not fulfill the criteria of the earlier recognized amyloid diseases. OBSERVATIONS--The unusual deposits, which were initially found in the dermis, were ultrastructurally composed of fibrillar material with a tubular substructure. Immunohistologically, the material was tested using a large panel of antibodies, and the results revealed that it was unlike any commonly known proteinaceous material. The deposits later spread to other organs and disturbed the vital functions of the body. CONCLUSIONS--We describe a unique syndrome characterized by fibrillar extracellular deposits that was recognized and differentiated from other similar clinical syndromes by ultrastructural examination. Further biochemical analysis is necessary to identify the origin of the material.

Fibrillar protein deposits with tubular substructure in a systemic disease beginning as cutis laxa. Publishing Authors By Initials

For similar integumentary system: skin research abstracts see: integumentary system: skin research

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Fibrillar protein deposits with tubular substructure in a systemic disease beginning as cutis laxa. Journal Published:

PUBLICATION TYPE: Research Support, Non-U.S. Gov

Journal: Archives of dermatology

VOLUME: 129

Page Numbers: 757-62

Journal Abbreviation: Arch Dermatol

ISSN: 0003-987X

DAY: 17

MONTH: Jun

YEAR: 1993

Fibrillar protein deposits with tubular substructure in a systemic disease beginning as cutis laxa. Information

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LANGUAGE: eng

NlmUniqueID: 372433

Fibrillar protein deposits with tubular substructure in a systemic disease beginning as cutis laxa. Keywords Mesh Terms:

KEYWORDS: Skin

MESH TERMS: ultrastructure

Chemical & Substance for Abstract: Fibrillar protein deposits with tubular substructure in a systemic disease beginning as cutis laxa. Information

Substance Name: Proteins

Registry Number: 0

Grant and Affiliation Information for Fibrillar protein deposits with tubular substructure in a systemic disease beginning as cutis laxa.

AFFILIATION: Department of Dermatology, Helsinki University Central Hospital, Finland.

Country: UNITED STATES

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MEDLINETA: Arch Dermatol

REFSOURCE: Arch Dermatol. 1994 Feb;130(2):256-7

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