Peripheral blood mononuclear cells (PBMC)
外周血單個核細胞(PBMC)
epidermolysis bullosa (EB)
表皮溶解水皰症(EB)泡泡龍
Very low levels of interferon-γ (IFN-γ), interleukin-1 (IL-1), and interleukin-2
(IL-2) were produced in vitro by PBMC from patients with severe forms of EB
PBMC在重度EB患者體外產生了極低水平的干擾素-γ(IFN-γ),白介素1(IL-1)和白介素2(IL-2)
epidermolysis bullosa (EB)
表皮溶解水皰症(EB)
Epidermolysis Bullosa Severity (BEBS)
表皮鬆解性大皰性嚴重度(BEBS)
However, the EB patients with a lower BEBS score showed significantly higher
IL-1β, IL-2, IL-6, IL-10, IL-12, TNF-β and IFN-γ than did the controls
(p = 0.015, p = 0.004, p = 0.004, p = 0.038, p = 0.038, p < 0.001
and p = 0.021, respectively).
但是,BEBS(嚴重)評分較低的EB患者的IL-1β,IL-2,IL-6,IL-10,IL-12,TNF-β和IFN-γ
明顯高於對照組(p = 0.015,p = 0.004,p = 0.004,p = 0.038,p = 0.038,p <0.001和p = 0.021)。
小結1:
由以上看來,泡泡龍症似乎缺少IL-1β,IL-2,TNF-β和IFN-γ。
IL-2-induced tumor necrosis factor (TNF)-β expression
IL-2誘導的腫瘤壞死因子(TNF)-β表達
(1)Epidermolysis bullosa simplex
is typically inherited in an autosomal dominant manner, affecting the keratin
genes KRT5 and KRT14.
通常以常染色體顯性方式遺傳,影響角蛋白基因KRT5和KRT14。
(2)Junctional epidermolysis bullosa
Junctional epidermolysis bullosa is an inherited disease affecting laminin and collagen.
是一種影響層粘連蛋白和膠原蛋白的遺傳性疾病。
(3)Dystrophic epidermolysis bullosa is caused by genetic defects (or mutations)
within the human COL7A1 gene encoding the protein type VII collagen (collagen VII).
營養不良性大皰性表皮鬆解是由編碼VII型膠原蛋白(膠原蛋白VII)
的人COL7A1基因內的遺傳缺陷(或突變)引起的。
(4)Epidermolysis bullosa acquisita
~~~
(4)Epidermolysis bullosa acquisita is caused by antibodies targeting type VII collagen
大皰性表皮鬆解症是由靶向VII型膠原的抗體引起的
(1)Epidermolysis bullosa simplex
IL-27 significantly increased KRT5 and KRT14 (Figure 4a).
IL-27顯著增加KRT5和KRT14(圖4a)。
IL-27 production is induced by TNF-a in human macrophages
IL-27的產生是由人巨噬細胞中的TNF-α誘導的
Under the same experimental conditions, IL-2 is seen to induce TNF-α mRNA.
在相同的實驗條件下,IL-2可以誘導TNF-αmRNA。
In this study, we report that TNF-α production is induced by IFN-γ treatment
在這項研究中,我們報告說IFN-γ處理可誘導TNF-α的產生
We now demonstrate that cooperative signaling induced by IFN-γ and LPS results in
the production of IL-27
我們現在證明,IFN-γ和LPS誘導的協同信號傳導導致IL-27的產生
In this study, we show for the first time that IFN-γ alone without any second
stimulus can induce IL-27p28 gene expression and IL-27 protein production
在這項研究中,我們首次顯示沒有任何第二刺激的單獨IFN-γ就能誘導IL-27p28基因表達和IL-27蛋白產生
小結2:
由以上可知,Epidermolysis bullosa simplex相當需要IL-2及IFN-r。
(2)Junctional epidermolysis bullosa
參考
泡泡龍症治療總整理(主要應該缺鋅維它命A、C,建議善存+維它命C+豆漿。)
(3)Dystrophic epidermolysis bullosa
interleukin (IL)-1beta up-regulate type VII collagen gene (COL7A1)
白介素(IL)-1beta(IL-1b)上調VII型膠原基因(COL7A1)
28.TNF-alpha and IL-1beta enhance the TGF-beta-mediated up-regulation of COL7A1 expression
in HaCaT keratinocytes
28.TNF-alpha(TNF-a)和IL-1beta(IL-1b)增強HaCaT角質形成細胞中TGF-beta介導的COL7A1表達的上調
The expression of IL-1beta protein was increased after IFN-gamma treatment
compared with the control group
與對照組相比,IFN-γ治療後IL-1β蛋白表達增加。
In this study, we report that TNF-α production is induced by IFN-γ treatment
在這項研究中,我們報告說IFN-γ處理可誘導TNF-α的產生
Under the same experimental conditions, IL-2 is seen to induce TNF-α mRNA.
在相同的實驗條件下,IL-2可以誘導TNF-αmRNA。
小結3:
由以上可知,Dystrophic epidermolysis相當需要IL-2及IFN-r。
(4)Epidermolysis bullosa acquisita
circulating IgA antibodies showing specificity against type VII collagen.
循環的IgA抗體顯示出對VII型膠原的特異性。
IFN-gamma down-regulates TGF-beta1-induced IgA expression
IFN-γ下調TGF-beta1誘導的IgA表達
小結4:
由以上可知,Epidermolysis bullosa acquisita相當需要IFN-r。
總結以上:
治療泡泡龍症主要靠IL-2及IFN-r,建議參考
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能提高IFN-γ的元素:
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