前情提要早衰症治療總整理(可能主要缺鋅、銅、錳,建議可以喝亞培安素草莓口味補充)
告訴我們:
Progerin,......及其表達被認為是導致核膜功能失調和早衰的原因。
在HGPS(早衰症)中,核纖層蛋白A的異常剪接和加工產生稱為progerin的蛋白質,
其誘導早期細胞衰老,其與增加的DNA損傷信號傳導相關。
積累的截短的法尼基化prelamin A形式,稱為progerin,是嚴重的早衰綜合症的標誌
lamin A is synthesized as a prelamin A precursor that undergoes an additional processing step catalyzed by the Zn metallopeptidase STE24 (ZMPSTE24) that removes the carboxy-terminal 15-amino-acid tail, including the modified cysteine to generate mature lamin A.
lamin A被合成為prelamin A前體,經過Zn(鋅)金屬肽酶STE24(ZMPSTE24)催化的額外加工步驟,去除羧基末端15-氨基酸尾部,包括修飾的半胱氨酸,以產生成熟的核纖層蛋白A.
Figure 1.
In Hutchinson-Gilford progeria syndrome cells, the second cleavage site in prelamin A is deleted, and this results in the accumulation of a permanently farnesylated and carboxy methylated prelamin A variant termed progerin.
在Hutchinson-Gilford早衰綜合徵細胞中,prelamin A中的第二個切割位點被刪除,這導致永久性法呢基化和羧基甲基化的prelamin A變體(稱為progerin)的積累。
Terminal cleavage of prelamin A is catalyzed by the zinc metallopeptidase ZMPSTE24, an enzyme that has recently been implicated in clearing proteins
prelamin A的末端切割由鋅金屬肽酶ZMPSTE24催化,該酶最近涉及清除蛋白質。
節錄自https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5074355/
The presence of progerin jeopardizes SIRT6 activation
progerin的存在危害SIRT6的活化
SIRT6 deficiency leads to defective genomic maintenance and accelerated aging similar to Hutchinson-Gilford progeria syndrome (HGPS)
SIRT6缺乏導致基因組維持缺陷和加速衰老,類似於Hutchinson-Gilford早衰綜合徵(HGPS)
Here, we report that lamin A is an endogenous activator of SIRT6 and facilitates chromatin localization of SIRT6 upon DNA damage.
在這裡,我們報導了核纖層蛋白A是SIRT6的內源激活劑,並促進SIRT6在DNA損傷時的染色質定位。
節錄自https://www.ncbi.nlm.nih.gov/pubmed/26549451
Box 2
Sirtuins are NAD+-dependent deacetylase and deacylase enzymes that control metabolism and ageing.
Sirtuins是NAD +依賴性脫乙醯酶和控制代謝和衰老的脫醯酶。
The nuclear SIRT6 is linked to telomere maintenance and genome stabilization. In mice, SIRT6 loss leads to genomic instability and premature ageing phenotypes, whereas overexpression of SIRT6 extends mouse lifespan54,55,60,62.
在小鼠中,SIRT6缺失導致基因組不穩定和過早衰老表型,而SIRT6的過表達延長了小鼠壽命。
節錄自https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5161407/
The structures revealed unique features of human SIRT6, including a splayed zinc-binding domain and the absence of a helix bundle that in other sirtuin structures connects the zinc-binding motif and Rossmann fold domain.
該結構揭示了人類SIRT6的獨特特徵,包括展開的【鋅】結合結構域和不存在螺旋束,其在其他sirtuin結構中連接鋅結合基序和Rossmann折疊結構域。
節錄自https://www.ncbi.nlm.nih.gov/pubmed/21362626
總結以上:
缺少鋅的金屬肽酶STE24(ZMPSTE24),prelamin A就無法成為成熟的核纖層蛋白A( lamin A )而成為progerin;而progerin又會危害SIRT6的活化加速衰老,但成熟的核纖層蛋白A( lamin A )則是 SIRT6的內源激活劑,SIRT6也含有鋅。
建議
建議可以喝草莓亞培安素或桂格特級完膳(無糖)補充。
建議從這裡
【KAWAI 卡歡喜】健康肝油球原味(300粒)
攝取維它命D+維它命A
含維生素A高的食物:胡蘿蔔、甜椒、鴨蛋黃、鵝蛋黃、枸杞、綠花椰菜、綠茶
又
Sirtuins是NAD +依賴性脫乙醯酶和控制代謝和衰老的脫醯酶
可能也需要補充維它命B3
含維它命B3高的食物(由多至少依序排列):蘑菇、香菇、花生、茶、南瓜