Advances in Clinical and Experimental Medicine

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Advances in Clinical and Experimental Medicine

2011, vol. 20, nr 5, September-October, p. 647–652

Publication type: review article

Language: English

AL Amyloidosis (Amyloidosis Antibody Light). Part 1. Definition, Classification, Amyloid Structure, Development and Etiopathogenesis of AL Amyloidosis

Amyloidoza AL (amyloidosis antibody light). Część I. Definicja, podział, budowa i rozwój amyloidu, etiopatogeneza amyloidozy AL

Lidia Usnarska-Zubkiewicz1,, Jadwiga Hołojda2,, Kazimierz Kuliczkowski1,

1 Departament of Hematology, Blood Neoplasms and Bone Marrow Transplantation, Wroclaw Medical University, Poland

2 Hematology Department of District Specialist Hospital of Legnica, Poland

Abstract

Amyloidosis is an inherited or acquired systemic storage disease in which a pathologic, amorphous substance produced as a result of abnormal protein metabolism and resistant to proteolysis is deposited in the extracellular space of various tissues (intracellular deposits occur rarely). This leads to the destruction of normal tissue architecture and disturbs its function. The development of amyloidosis is associated with the transformation of the α-spiral conformation of fibrous protein into a stratified, parallel, folded spatial β-conformation. The transition of the fibrous proteins conformation and their tissue localization causes resistance of the fibers to proteolytic processes as a result of a limited access of the protein converting enzymes. In this paper we show the actual classification of amyloidosis and up-to-date knowledge about the amyloid structure, development and etiopathogenesis of AL amyloidosis.

Streszczenie

Amyloidoza (skrobiawica) jest wrodzoną lub nabytą układową chorobą spichrzeniową, w przebiegu której dochodzi do gromadzenia się w przestrzeni pozakomórkowej różnych tkanek (rzadko wewnątrzkomórkowo), patologicznej, amorficznej substancji, która powstaje na skutek nieprawidłowego metabolizmu białek i jest oporna na proteolizę. Prowadzi to do zniszczenia normalnej architektoniki tkanek i zakłócenia ich funkcji. Rozwój amyloidozy jest związany z przekształceniem α-spiralnej konformacji włókien białkowych w warstwową, równoległą, pofałdowaną, przestrzenną β-konformację. Zmiana konformacji włókien białkowych i ich tkankowe umiejscowienie powoduje oporność włókien na procesy proteolityczne w wyniku ograniczonego dostępu enzymów rozkładających białka. W pracy przedstawiono aktualny podział amyloidozy oraz obecny stan wiedzy na temat rozwoju, budowy i etiopatogenezy amyloidozy z łańcuchów lekkich.

Key words

amyloidosis AL, classification, amyloid structure, etiopathogenesis

Słowa kluczowe

amyloidoza AL, klasyfikacja, struktura amyloidu, etiopatogeneza

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