Liver Inclusions in Erythropoietic Protoporphyria

Wolff, Klaus; Wolff-Schreiner, Elisabeth C.; Gschnait, F

doi:10.1111/j.1365-2362.1975.tb00424.x

Cited by 41 publications

(16 citation statements)

References 18 publications

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“…8) as well as in tissue fixed in aldehydes. The straight profiles had morphological characteristics similar to those reported for protoporphyrin IX crystals in hepatocytes from patients with erythrohepatic protoporphyria (Matilla and Molland, 1974;Klatskin and Bloomer, 1974;Wolff et al, 1975;Bruguera et al, 1976) and in mouse hepatocytes following drug-induced protoporphyria (Waterfield et al, 1969;Matilla and Molland, 1974;Gschnait et al, 1975). Such crystals were extracted from porphyric mouse livers and found to have the solubility and absorption characteristics of protoporphyrin IX; upon recrystallization, their ultrastructure was identical to that of known (commercial) protoporphyrin IX crystals .…”

Section: Relationship Of the Trilaminar Forms To Porphyrinsmentioning

confidence: 50%

Ultrastructural visualization of intracellular porphyrin in the rat Harderian gland

Carrière

1985

Anat. Rec.

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The Harderian glands of male Albino rats 1-24 months of age were studied by electron microscopy. In most glands, a few acinar cells contained straight and curved trilaminar profiles identical in form to the material in the luminal masses of porphyrin pigment. They resembled the structures which several investigators have identified as crystals of protoporphyrin IX in porphyric human and mouse hepatocytes. Protoporphyrin IX is the predominant form synthesized by rat Harderian cells in vitro. The trilaminar profiles were within the cytoplasm but not within the lipid secretory vacuoles. A large number of the acinar cells had finely tubular endoplasmic reticulum (ER). However, cells containing the trilaminar forms consistently had dilated ER vesicles. This change may have been a prelude to cell death, for some pigment-containing cells attached to the acinar basal lamina also displayed fragmented organelles and a loss of density of the cytoplasmic matrix. In some acinar lumina there was abundant cell debris along with the trilaminar profiles. It is concluded that in the rat, some of the Harderian free porphyrins can be released through cell death.

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Section: Relationship Of the Trilaminar Forms To Porphyrinsmentioning

confidence: 50%

Ultrastructural visualization of intracellular porphyrin in the rat Harderian gland

Carrière

1985

Anat. Rec.

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“…LCs in situ or freshly isolated from the epidermis have some capacity to endocytose (8,33,34). They are equipped with acidic organelles like endosomes (10), the necessary cellular compartments for antigen processing (11,12).…”

Section: Biochemistry Of Class II and Ii Molecules Of Immaturementioning

confidence: 99%

Class II major histocompatibility complex molecules of murine dendritic cells: synthesis, sialylation of invariant chain, and antigen processing capacity are down-regulated upon culture.

Kämpgen

Koch

et al. 1991

Proc. Natl. Acad. Sci. U.S.A.

146

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Dendritic cells (DCs), such as Langerhans cells (LCs) of the epidermis and the DCs of lymphoid organs such as spleen, are potent antigen presenting cells. DCs express high levels of major histocompatibility complex (MHC) class I molecules, but, partly because of the low numbers of primary DCs in any tissue, there has been no detailed study of the biochemistry of their class II molecules. This information may be needed to help explain recent findings that DCs process native protein antigens when freshly isolated from epidermis and spleen. Processing ceases during culture, yet a strong accessory function for activating resting T cells develops. We studied immunoprecipitatts of DC class II and invariant chain (1,) molecules by two-dimensional gel electrophoresis. We found that (i) freshly isolated LCs synthesize large amounts of class II and I, polypeptides; (ii) I; molecules that are known to be involved in antigen processing display an unusually large number of sialic acids in fresh LCs; (iii) with culture, class II and I, synthesis decreases dramatically and has virtually ceased at 3 days; and (iv) the turnover of class H in pulse/chase experiments is slow, being undetectable over a 12-to 32-hr culture period, whereas the turnover of I, is rapid. We conclude that MHC class H molecules of DCs do not seem to be qualitatively unique. However, the regulation of class II and Ii expression is distinctive in that biosynthesis proceeds vigorously for a short period of time and the newly synthesized class H remains stably on the cell surface, whereas Ii turns over rapidly. This may enable DCs to process and retain antigens in the peripheral tissues such as skin and migrate to the lymphoid organs to activate T cells there.Dendritic cells (DCs) represent a system of abundantly major histocompatibility complex (MHC) class II-expressing leukocytes. They occur in nonlymphoid organs, blood, afferent lymph, and lymphoid tissues (1). Two states ofdifferentiation can be distinguished (2, 3). "Immature" DCs, exemplified by freshly isolated epidermal Langerhans cells (LCs), are weak stimulators of resting T cells both in the allogeneic mixed leukocyte reaction (4,5) and in polyclonal responses such as oxidative mitogenesis, concanavalin A mitogenesis, and anti-CD3 mitogenesis (6). These fresh LCs (7, 8) as well as fresh DCs from spleen (9), however, are efficient in processing native protein antigens for MHC class II-restricted presentation to presensitized peptide-specific T cells (in vivo-primed T cells, clones, T-T hybridomas). Upon 1-3 days of culture in macrophage-or keratinocyte-conditioned medium, or in culture medium supplemented with granulocyte/macrophage colony-stimulating factor, their functional properties become inverted. "Mature" DCs lose the capacity to process exogenous antigens but at the same time acquire the ability to sensitize resting T cells (2, 3). The loss ofprocessing function is paralleled by the loss of acidic organelies such as endosomes (10), which are known as the compartments where class II e...

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“…This study used ESR with a surface resonator to detect in vivo ROS generation with light exposure in the skin of griseofulvin-induced protoporphyria model mice, a standard model for EPP Konrad et al, 1975;Wolff et al, 1975;Plosch et al, 2002).…”

mentioning

confidence: 99%

In Vivo Oxygen Radical Generation in the Skin of the Protoporphyria Model Mouse with Visible Light Exposure: An L-Band ESR Study

Takeshita

Takajo

Hirata

et al. 2004

Journal of Investigative Dermatology

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Although oxygen radicals are thought to play a key role in the skin injury that is caused by protoporphyria, there is no direct evidence of generation of these radicals in vivo. This study measured the generation of oxygen radicals caused by visible light non-invasively in the skin of griseofulvin-induced protoporphyria model mice, using an in vivo electron spin resonance spectrometer equipped with a surface-coil-type resonator that could detect radicals within about 0.5 mm of the skin surface. A durable nitroxyl radical was administered intravenously as a probe. Light irradiation enhanced the decay of the nitroxyl signal in griseofulvin-treated mice, whereas light irradiation did not enhance the signal decay in control mice. The enhanced signal decay was completely suppressed by intravenous administration of hydroxyl radical scavengers, superoxide dismutase or catalase, or the intraperitoneal administration of desferrioxamine. The enhanced signal decay with illumination was reversible, and quickly responded to turning the light on and off. These observations suggest that the hydroxyl radical is generated via an iron-catalyzed reaction in the skin. This paper demonstrates, for the first time, the specific generation of oxygen radicals in response to light irradiation of the skin of protoporphyria model mice.

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Liver Inclusions in Erythropoietic Protoporphyria

Cited by 41 publications

References 18 publications

Ultrastructural visualization of intracellular porphyrin in the rat Harderian gland

Ultrastructural visualization of intracellular porphyrin in the rat Harderian gland

Class II major histocompatibility complex molecules of murine dendritic cells: synthesis, sialylation of invariant chain, and antigen processing capacity are down-regulated upon culture.

In Vivo Oxygen Radical Generation in the Skin of the Protoporphyria Model Mouse with Visible Light Exposure: An L-Band ESR Study

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