Inhibition of glycosaminoglycan synthesis using rhodamine B in a mouse model of mucopolysaccharidosis type IIIA.
糖胺聚醣合成的抑製作用使用羅丹明B在小鼠模型粘多醣類IIIA部。
_原文位置http://www.ncbi.nlm.nih.gov/pubmed/16857766
Department of Genetic Medicine, Children, Youth, and Women''s Health Service, North.
Reduction of an enzyme activity required for the lysosomal degradation of glycosaminoglycan (gag) chains will result in a mucopolysaccharidosis (MPS) disorder. Substrate deprivation therapy (SDT), a potential therapy option for MPS with residual enzyme activity, aims to reduce the synthesis of gag chains, the natural substrate for the deficient enzyme. Reduced substrate levels would balance the reduced level of enzyme in patient cells, resulting in normalized gag turnover. Rhodamine B, a nonspecific inhibitor, reduced gag synthesis in a range of normal and MPS cells and also decreased lysosomal storage of gag in MPS VI (72%) and MPS IIIA (60%) cells. Body weight gain of male MPS IIIA mice treated with 1 mg/kg rhodamine B was reduced compared with untreated MPS IIIA mice and was indistinguishable from that of normal mice. Liver size, total gag content, and lysosomal gag was reduced in treated MPS IIIA animals as was urinary gag excretion. Lysosomal gag content in the brain was also reduced by treatment. The alteration in MPS IIIA clinical pathology by rhodamine B, combined with the observation that treatment had no effect on the health of normal animals, demonstrates the potential for SDT in general as a therapy for MPS disorders.
PMID: 16857766 [PubMed - indexed for MEDLINE]
Matthew (6 1/2, MPSIIIA),
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2007年4月9日，下午11點58
鑑於剛才的信息我送給她的addt''l資料河畔她能夠發現葡萄糖不是一個好的替代醫學使用至少對於我們
誰不正確打破硫酸乙酰肝素的下跌葡萄糖艾滋病在建立了這一點。 請問MPS3打破硫酸乙酰肝素正常？
我知道她說，在某些情況下，在MPS的這將是安全的，但是沒有MPS的我和其他有缺陷在這......
I hope this makes sense.....我希望這是有道理的..... ..當我們問我的遺傳學家，他同意，並解釋了一些更
起初他以為是安全的，直到我的氣相色譜指出，艾滋病在建立的H /硫酸，然後，他像我wouldnt建議。
我做了大量的GC這一研究雖然...
埃麗卡
MPS I Scheie, Age 25
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200707年4月10日，上午06點47分
我想找到您的張貼文章關於此埃麗卡，因為我覺得這個問題與葡萄糖，是因為輔助生產硫酸皮膚素，
而不是硫酸乙酰肝素。的一切我已經閱讀葡萄糖並不助手生產硫酸乙酰肝素，但協助生產硫酸皮膚。
MPS3孩子無法代謝硫酸乙酰肝素，但沒有任何硫酸皮膚素問題。
Sheila 希拉
MPS IIIA 14歲
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April 10th, 2007, 01:53 PM 07年4月10日，下午1時53分
希拉，你說得對，那是我的錯，我要看看我能找到這個議題。 感謝您指出了這一點！
埃麗卡
MPS I Scheie, Age 25
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April 10th, 2007, 03:40 PM 07年4月10日，下午3點40分
引言:該用什麼劑量的氨基葡萄糖鹽酸 2008年3月
Hi everyone,您好大家， 薩沙的母親 星期六2008年3月22日下午4時02分
Well, I went to the market today for Easter and also went looking for a bottle of glucosamine Hcl.
好吧，我去了當今市場的復活節，也開始尋找一瓶葡萄糖鹽酸。
I found it, it''s a no name brand but just for now I wanted to get Sasha started on SOMETHING as opposed to nothing at all.
我覺得，這是一個沒有品牌，只是現在我想的東西薩莎開始，而不是一無所知。
I hope to graduate soon to a better brand!
我希望畢業後到一個更好的品牌！
I am just curious, how much should I give her?
我只是好奇，有多少，我應該給她？ She''s 9 3/4 years old and weighs about 60 lbs.
她的9又3 / 4歲，體重大約60磅。
The tablet I got is 600 mg I believe, and it''s quite large and easy to cut in half and crush.
我的片是600毫克，我相信，而且相當大，容易減少一半和愛戀。
Thanks for any input!感謝您的任何投入！
Joanne~Mom to Sasha喬安妮〜母親薩莎
he dose we give Jesse is 1000mg.
我們給予的劑量是1000mg傑西。
He was weighing about 65 lbs or so when I started at that dose.
他重約六五英鎊或因此，當我開始在這個劑量。
I did see a difference in his ability to focus for longer on doing things.
我沒有看到他的能力的差異將重點放在長期的事情。
We too were headed to 2000 mgs, but since I saw results at 1000mg, I never went any higher.
我們也正朝著到2000mgs，但因為我看到的結果1000mg ，我從來沒有任何更高。
He weighs 120 lbs now and still gets 1000mgs.
他現在體重一百二十〇磅仍然得到1000mgs 。
The bottle does suggest 2000mgs daily on ours for an adult,
瓶子不建議2000mgs每天我們成人，
So I guess I could up it, but I don''t see any need to at this time.
所以我想我可以了，但我看不出有什麼必要在這個時候。
Sheila said,希拉說，
Quote:
There are many doctors who now believe a big component of the damage caused in MPS is inflammation.
有許多醫生認為，現在誰一大組成部分造成的損害是公安部炎症。
We also give other supplements that are good for inflammation and also antioxidants too.
我們還讓其他補充劑的是良好的抗炎和抗氧化劑也太多。
Keep us posted if you see any changes over the next 2 weeks.
讓我們張貼如果你看到任何變化，在未來2個星期。
I noticed changes in Jesse in about 4 - 5 days, and so did school.
我注意到變化傑西在約4 - 5天，所以沒有上學。
We never tell them when we add a supplement,
我們從來不告訴他們，當我們購買的一種補充，
we use their responses if any, as evidence if something is working,
我們會使用他們的答复如果有，作為證據的東西有作用，這樣
that way it''s not just our imagination..
，這不僅是我們的想像力..
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Hi everyone,您好大家， 2008年3月23日上午09時58分
Thank you for that input, I kind of had no idea where to start, and now I do!!
謝謝你的投入，我種不知道從哪裡開始，現在我做！
I gave Sasha a half tablet yesterday, which would have been 300 mg''s.
我給了薩沙半片昨天，本來300毫克的。
It sounds OK to at least give her one tablet, and even one and a half tablets would be just 900 mg''s.
這聽起來確定至少給她一個片劑，甚至一個半片會是900毫克的。
So I feel good knowing it is OK to go more than the half tablet.
所以，我感覺很好知道這是行去超過一半片劑。
Thank you again, very much!再次感謝你，非常！
Joanne~Mom to Sasha喬安妮〜薩莎的母親
Another FYI - you may want to make ABsolute sure Sasha has MPS III and not another type
bc atleast MPS I does not break down the main supplement that is in Glucosomaine - it is safe
in Sanfillipo but in other MPS''s like MPS I we do already store the Dermatan/heparan sulfates
and so this can be counter-effective.
We give 1400 mgs.我們給予1400mgs。
700 in the morning and 700 in the evening.
700在上午和晚上的700 。
I did increase but didn''t see any benefits over this.
我沒有增加，但沒有看到任何的好處在此。
I did see behavioural improvements at the time and I''m sure it''s helping against inflamation.
我沒有看到行為改善的時候，我相信這是幫助對炎症。
I also take them myself.我也把他們自己。
Sebastien has been on glucosamine since he was 2 1/2 .
塞巴斯蒂安一直葡萄糖，因為他是2 1 / 2 。
He is now taking 1000 mg in the morning in his juice or milk.
他現在正在1000毫克上午在他的果汁或milk.
We buy the capsule form.We just open the capsule and off we go.Sebastien is now 7 1/2 and is 76 pds.
We購買膠囊form.We只需打開返回艙和關閉，我們go.Sebastien現在7 1 / 2和76 pds.
We truly feel it has helped him with his joints and range of motions.
We真正感受到它幫助他與他的關節和各種動議。
Dr Clarke always mention how he is surpise to see Sebastien range of motion
郭大偉博士總是提到他是如何看到塞巴斯蒂安surpise一系列議案，
with his elbow and shoulders it is almost normal .Is it the glucosamine ?
他的手肘和肩膀幾乎是正常的。難道葡萄糖？
who knows but it has not hurt him so for us we feel it was and still is worth it.
誰知道，但它不會傷害他，所以對我們來說，我們認為它仍然是值得的。
Anne and Sebastien安妮和塞巴斯蒂安
I met Dr. Poplawski at the International MPS Symposium in Germany in 2004.
我會見了博士Poplawski國際研討會在德國公安部於2004年。
She said that about half the children showed improvement and she was looking for more candidates for trials but Daniel was too far away.
她說，大約一半的兒童有進步，她正在尋找更多的候選人進行審判，但丹尼爾是太遙遠。
In her talk she implied that 50% was not that successful, and that more trials were needed.
在她說話，她暗示 ， 50 ％沒有成功，而且還需要更多的審判。
Maybe she just didn''t receive any more financing for the project.
也許她只是沒有得到任何更多的研究經費
Thank you for the link Elizabeth!!謝謝你的鏈接伊麗莎白！
You know I am also curious if the 50% with improvement had any diet changes or where given other supplements as well
你知道我也很好奇， 50 ％與飲食習慣的改善有任何變化或給予其他補充品，以及 。
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黛布拉 [星期二2008年3月25日
Studies are usually pretty rigorous about controlling for other possible changes.
研究通常是相當嚴格的關於控制其他可能發生的變化。
My assumption would be that the kids weren''t allowed to have any other dietary or supplementation changes during the study.
我的假設是，孩子們不允許有任何其他食物或補充變化的研究。
Also, 50% is not a great statistic, but it''s better than nothing.此外，
50 ％是不是一個偉大的統計，但總比沒有好。
It''s not like we have a plethora of treatment options available to us...
它不象我們有太多的治療方案提供給我們...
Dr. Poplawski is looking for a couple children to finish the Sanfilippo study in Toronto.
博士Poplawski正在尋找一對夫婦兒童完成MPS的研究在多倫多。
If anyone is interested please contact her at
如果有人有興趣，請聯繫她
nicola.poplawski@sickkids.ca
I spoke with Jesse''s Dr. after learning about the study through you.
我與傑西的博士學習後，通過你的研究。
He checked into Dr. Clarke studies, and we decided to try our own on Jesse as well.
他住進克拉克博士的研究，我們決定傑西嘗試。
He has been on Glucosamine Hydrochloride since Feb 13th, 2004.
他用氨基葡萄糖鹽酸鹽自2004年2月13號。
We didn''t tell school about it.我們沒有告訴學校的。
But after about 8 days of being on it.但是，大約8天之後，被它。
School notified us to tell us how well Jesse was doing and how much more attentive he''s been and interested in the lessons.
學校通知我們，告訴我們如何做以及傑西以及如何更周到的他已經和感興趣的教訓。
(so we told them then about the glucosamine) He also taps a lot less and chews a lot less.
（所以我們告訴他們關於葡萄糖）他也少了很多水龍頭和咀嚼大為減少。
He responds a little more readily than before and much less whinny.
他回答說一點比以前更容易，更whinny 。
So all in all so far, it has been a wonderful thing.
因此，所有的一切，到目前為止，它一直是美妙的事情。 Oh..噢..
his legs also do not go to sleep on the bus ride home anymore.
他的腿還沒有睡覺就乘車回家了。
The bus aide said he is much more agile about getting up and down to go on and off the bus.
巴士助手說，他更敏捷約越來越向上和向下去和下車。
Where as many days he was like a poor little ol''e drunk man and couldn''t walk for about 3 -5 minutes at least.
凡多天，他像一個可憐的小ol''e喝醉酒的人，不能步行約3 -5分鐘至少。
We had to rub his legs to get circulation going.
我們不得不擦他的腿讓循環下去。
This sounds wonderful Jesse''s Mum.
這聽起來美妙傑西的媽媽。
I''ve also just started trying the Glucosamine Hydrochloride
(ordered 500 mg caplets from the States).
我也剛剛開始嘗試的氨基葡萄糖鹽酸鹽（ 500毫克粒排列的國家） 。
Trouble is I''ve no idea how much to give him and when.
麻煩的是我不知道有多少給他當。
What are you doing?您在做什麼？
Are you also seeing any improvement in the speech?
你還看不到任何改善的講話？
Lots of love and good wishes 很多熱愛和良好祝愿
丹尼爾（ MPSIIIA出生1月91 ）
丹尼爾的網站： http://www.caringbridge.org/visit/danielschuepbach
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Glucosamine - 葡萄糖- March 5th, 2004, 10:53 PM 2004年3月5日，下午10時53分
Hi Elizabeth,您好伊麗莎白
I sent you an e-mail, but I am givng Jesse currently 1000mg once a day.
我給您發送一封電子郵件，但我givng傑西目前1000mg ，每日一次。
he weighs 76 lbs. I have seen a difference in speech, he is saying more than he has in the last 8 monhts,
體重七十六磅。我看到了不同的講話，他說他已經超過了去年8 monhts ，
but nothing like he was 2 years ago.
但什麼也沒有想他是2年以前。
But there is much improvement.
但仍然有很多改進。
If he does well over the next 25 days or so, we are going to reduce the dose to 500mg,
如果他這樣做以及在未來25天左右的時間，我們要減少劑量500 ，
and see if he maintaiins over 30 days on 500 mg, the same.
看看他maintaiins在30天內對500毫克，同樣的。
If he doesn''t then we will go back to 1000mg.
如果他不那麼我們將回到1000mg 。
But he is much more content and more focused on everything, yet he is not in a sedated state of mind at all.
但他更內容和更集中於一切，但他卻沒有音信的精神狀態在所有。
No whining, less chewing, and tapping, but still active and more movement in his hips and leg joints than before.
沒有抱怨，減少咀嚼，和竊聽，但仍積極和更運動在他的臀部和腿部關節比以往任何時候。
He hasn''t been stiff at all after about the first 8 -10 days.
他沒有被僵硬後，在所有的前8 -10天。
I love watching him run and go up and down steps.
我喜歡看他運行和上下的步驟。
He was actually starting to give up steps before I started him on glucosamine,
他實際上是開始放棄步驟之前，我開始了他對葡萄糖，
and he couldn''t hardly walk after sitting for 30 minutes or more in a car or on the bus.
他不能坐在後幾乎步行30分鐘或以上的汽車或巴士上。
Now he''s doing stairs again with some confidence, as long as he has a handrail,
現在，他再次做樓梯的一些信心，只要他有一個扶手，
and has not been stiff at all, even after long bus or car rides
並沒有在所有僵硬，即使經過長時間搭乘巴士或汽車
Keep me posted on your experiences, I love to hear the reports.
回執上的經驗，我愛聽取報告。
Patty帕蒂
God never gives me more than I can handle, though at times I wish the load was lighter :-)
上帝給了我從來沒有比我更可以處理，但有時我想是的負載較輕:-)
Love to you all! 愛大家！
Hi Elizabeth.您好伊麗莎白。
I apologise for not responding to your post sooner.本人道歉沒有回應您的帖子越快。
Jesse''s mom pretty much said it all.傑西的媽媽說，幾乎所有。
The only difference is Sarah takes 1500mgs per day.唯一的區別是薩拉考慮1500mgs每天。
The most exciting for us was the size of Sarah''s liver.最令人興奮的是，我們的規模Sarah的肝臟。
It was very large at the beginning of the glucosamine trial.這是非常大的開頭葡萄糖治療。
The size has now lessened significantly.現在的規模已經大大減少。
Before Sarah began, she had become very still and quiet, making no sounds or eye contact ,
莎拉開始之前，她已經變得非常寂靜，安靜，沒有任何聲音，或使眼神接觸，
with in a few weeks she was running around laughing and smiling, she even said a couple of words.
並在幾個星期後，她四處笑著和微笑，她甚至說了幾個單詞。
She is able to focus on her toys more and more all the time.
她是能夠專注於她的玩具越來越多的所有的時間。
She actually plays with them instead of ignoring or throwing them.
她與他們實際發揮不是忽視或投擲它們。
Her Teacher is very pleased with the progress she has made in school.
她的老師是非常的進展感到高興，她已在學校。
Sarah is in a sensory integrative, cause-effect program that requires much co- operation.
薩拉是在感覺統合，因果計劃，需要大量的合作。
She is doing well at this time.她是做好在這個時候。
I often go to the school just to watch her experssion when she eg.
我經常去學校只是為了看她時，她如experssion 。
makes the lights blink or or turns the knob so she can hear the music.
使燈光閃爍或或轉動旋鈕，讓她能聽到的音樂。
It is so precious.它是如此珍貴。
She was also having trouble swollowing.她也遇到了問題swollowing 。
She was mainly eating puree food.她主要是吃醬食品。
Now there isn''t anything she doesn''t eat.現在有沒有什麼她不吃飯。
Sarah sleeps through the night, without any meds.莎拉通過晚上睡覺，沒有任何meds 。
The only medication she take is Tegratol.唯一的治療，她採取的Tegratol 。
If there are any other specifics I could help with, please email me.
如果有任何其他的細節我可以幫助，請給我發電子郵件。
Kathie and Sarah凱茜和薩拉
薩拉11歲MPS3B
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March 9th, 2004, 08:22 AM 2004年3月9日，上午08時22分
We started Andrew yesterday on the medicine.
我們開始安德魯昨天就藥品。
Of course we don''t know what he is taking.當然我們不知道他要。
Even if we got the placbo it was well worth the trip to be able to help other MPS3 children.
即使我們獲得了placbo它是值得的訪問能夠幫助其他MPS3的兒童。
Dr. Clarke and Dr. Poplawski are very confident in this study.
克拉克博士和博士Poplawski非常有信心在本研究中。
They are wonderful doctors.他們美好的醫生。
Andrew is taking 3.2 ml of the powdered form mixed with water.
安德魯正在三點二毫升的粉末與水混合。
Not sure what that is in mgs.不知道這是在MGS。
Will keep you posted.會及時向大家發布。
Paulina寶琳娜
安德魯公安部三B
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March 12th, 2004, 02:37 PM 2004年3月12號，下午2時37分
Good afternoon everybody大家下午好
I brought the question to Dr Joe Clarke,he is the doctor who follows Sebastien at Sick Kids .
我把這個問題向克拉克博士喬，他是醫生誰如下塞巴斯蒂安在患病兒童。
We have started Sebastien on 750mg of glucosamine daily ,he weighs 72pds and has severe Hunters .
我們已經開始塞巴斯蒂安在每日750mg氨基葡萄糖，體重72pds ，並已嚴重獵人。
The caplets are easy to brake in 1/2 and I crunch該粒容易制動器在1月2日，我緊縮
it up in his juice or meal.它在他的果汁或晚餐。
I have not seen a huge difference yet but it has only been 3 weeks :fluffy
我還沒有看到一個巨大的差異，但是它只能三週：蓬鬆
Sebastien does not speak but his actions and laughter are how we can judge what works ,
塞巴斯蒂安沒有說話，但他的行動和笑聲是我們如何才能判斷是什麼在起作用
so lets hope we can，所以希望我們能夠讓
see some improvements not matter how big or small it might be Best of luck to you
看到一些改善沒有多大的問題或小最好的你好運
Anne安妮
Keep hope close to your heart,保持希望貼近您的心，
as a cupped hand protects a flame作為一個杯狀手保護的火焰
Sebastien has severe Hunter syndrome塞巴斯蒂安已經嚴重亨特綜合徵
http://www.caringbridge.org/visit/sebastiencairns
t''s great to hear that you were able to speak to Dr. Clarke about it, I hope you get as much results as I did.
很高興聽到你能克拉克博士說了，我希望你得到了結果，我做到了。
It has really been a big difference for Jesse.它確實是一個很大的區別的傑西。
He has Sanfilippo A and school has really been impressed with the changes,
he scored a 100% in all his IEP assessments this quarter.
他認為MPS3A和學校有真正的變化留下了深刻印象，他取得了100 ％ ，
所有的個人化教育計劃評估本季度推出。
That is a first for him :cheers:這是第一次為他：歡呼：
Keep us posted on Sebastien''s progress.讓我們張貼在塞巴斯蒂安的進展。
It would be great if helps out Hunter too.這將是巨大的，如果亨特也幫助了。
Patty and Jesse帕蒂和傑西
Patty and Jesse 帕蒂和傑西
MPS III A 公安部三A
http://www.caringbridge.org/md/myjess
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big improvements - 重大改進- March 16th, 2004, 07:01 PM 2004年3月16日，下午7時01分
Wow I really feel like jumping of joy! 哇我真的覺得自己跳的喜悅！
Sebastien has been on 750mg of glucosamine daily ,4 weeks to the day !
塞巴斯蒂安一直在氨基葡萄糖每日750mg ， 4週來的每天！
What an amazing difference since Sunday :Party: 什麼驚人的差異 ， 因為週日： Party
Even though he has a really bad cold ,
儘管他有一個非常糟糕的冷，
he has been in an amazing mood ,
他一直在一個驚人的心情，
paying attention for longer periods of time,he has been participating more ....
關注時間較長，他一直參與更多....
At daycare they have started him on a picture exchange program 3 weeks ago
在日托，他們已開始他的圖片交流計劃3週以前
and never really caught on ,but today it clicked!
和從未真正流行，但今天它點擊！
The girls who take care of him at day care were just so happy!
女孩誰照顧他在日托只是太高興了！
They took pictures of him and also filmed him while he was showing off
他們拍攝了他和他的同時也拍攝他炫耀 :
I dont expect him to start talking but it is amazing how nice it is to see some improvements no matter how small it is!
我不期待他能開口說話，但令人驚奇的是如何很好地看到了一些改進不管多麼小啊！
Thanks for letting me brague a bit!
感謝您讓我brague一點！
Ciao Anne Ciao的安妮
Keep hope close to your heart,保持希望貼近您的心，
as a cupped hand protects a flame作為一個杯狀手保護的火焰
塞巴斯蒂安已經嚴重亨特綜合徵
http://www.caringbridge.org/visit/sebastiencairns
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April 3rd, 2004, 11:37 AM 2004年4月3日，上午11時37分
We have been in the study for a month now.我們一直在研究了一個月，現在。
We believe that we have the placbo, we have not seen any changes in Andrew''s behavior.
我們認為，我們有placbo ，我們沒有看到任何變化安德魯的行為。
It''s a little disappointing, but it was the chance we took.
它感到有點失望，但我們的機會了。
Hopefully all the results will come in and we can use it for all MPS children.
希望所有的結果將在我們可以利用它為所有MPS兒童。
Paulina寶琳娜
安德魯MPS3B
Many hugs and prayers, 許多擁抱 ， 並為他們祈禱，
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April 3rd, 2004, 12:36 PM 2004年4月3日，下午12時36分
To answear your question Sharon you do not have to be part of the study ,
為了您的問題，沙龍answear你不必是研究的一部分，
like everything else I always bring things up to Dr Clarke his genetic doctor befor giving hoim anything new
像其他一切事情我總是帶來高達克拉克博士醫生befor他遺傳給hoim什麼新
I purchase the glucosamine at the drug store or health food store
購買的葡萄糖在藥店或健康食品店
Paulina when the study is finish if in fact he was on a placebo you know
you can try it and hopefully you will see some improvement
保時完成這項研究，如果事實上他是安慰劑你知道你可以嘗試，並希望你會看到一些改善
Good luck祝您好運
Anne安妮
Congratulations Anne, I''m so glad your''e seeing results.
恭喜安妮，我很高興your''e初見成效。
It''s so nice when we can see any change at all for the better.
這是太好的時候 ， 我們可以看到有任何改變都變得更好。
My next experiment with Jesse is with the Mangosteen Fruit.
我的下一個實驗傑西是與山竹果。
It seems to have some amazing qualities. 這似乎有一些驚人的素質。
My neice became a distributor and after doing some research on it myself,
I decided to try Jesse and the rest of us on the Magonsteen Juice drink "XanGo".
我的侄女成為分銷商和一些研究後，做它自己，我決定嘗試傑西和我們其他人的
Magonsteen果汁飲料“台灣XanGo ” 。
There''s a Dr. Templeman who has a web site with some very good information of the stuff and it''s all juice.
有一個博士坦普爾曼誰擁有一個網站，與一些非常好的信息的內容和它的所有果汁。
It''s at http://www.mangosteenmd.com/
It will most likely take a month to see any results if there are any,
but I will be optimistic and curious of course.
這將最有可能採取每月不到任何結果是否有，但我將樂觀，好奇的課程。
I will keep you all posted if anything happens good or bad after being on it.
我將隨時向你所有張貼在任何情況下好消息還是壞消息後，它。
If it does any good, I just may become a distributor myself..
如果沒有任何好處，我只可能成為經銷商自己..
安德魯 11 MPS III B G 管式分流
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2009.02.20
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