maple syrup disease的八卦，Yahoo名人娛樂都在討論

MY NUTTY ACAI BOWL 🤤
If you are craving for some sweet, create your own, play around with natural ingredients such as sweet potatoes, pumpkins, oats , frozen fruits and create some deliciousness using organic nuts butter, dark chocolate, raw honey, maple syrup, coconut, chia seeds, açaí, cacao nibs and so on..
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Refined sugar is one of the worst ingredient increasing risk of obesity, type 2 diabetes, heart disease, liver disease and some type of cancers and also surprisingly linked to depression. Unfortunately you don’t only find it in sweet products but also in most of processed food.
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I’m myself a sweet tooth, i would chose desserts over anything.. lava cake, cinnamon rolls, caramel ice-cream, tiramisu, apple pie... 🤯🤤 but a healthy diet as everything, should be well balanced. Be good to yourself and be aware of what you are consuming 👍🏼
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Açaí by @tropicalbite_acai
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#acai #acaibowl #homecooking #nutrition #healthylifestyle #healthydiet

《毒荔枝之謎大揭密》轉一篇崇拜的學長的最新分享，Lowei的一句白話文簡譯就是：不要只吃(生)荔枝而沒吃飯飯唷！

#遺傳代謝之不思議 07話 毒荔枝之謎全揭祕

**本期內容比較複雜，歡迎來聆聽我的UGY代謝學**
這兩天的勁爆話題是吃生荔枝會導致低血糖，故事綱要請見超認真學弟 馬聖翔 整理的 #TINYNOTES，大抵是印度一個靠近尼泊爾的Muzaffarpur縣每逢五、六月的荔枝盛產期，當地的孩童常在清晨發生低血糖與急性癲癇發作。(語言學小解釋：興地語的-pur來自印歐語系的-pura，為「港口」之意，亦如-port, -furt, -ford)後來在未成熟的荔枝當中發現高濃度的次甘胺酸A(Hypoglycin A, 另一種次甘胺酸B毒性較低)與methylene cyclopropyl acetic acid(MCPA)，小朋友就是吃了過量的生荔枝以為飽餐一頓，沒再吃其他東西就上床睡覺，所以導致凌晨低血糖。但通常單就血糖低並不會造成腦子壞掉；進一步分析，他們的尿液有機酸出現了不正常的代謝物，研判是異常代謝物和低血糖合併引發腦病變。

次甘胺酸A在人體中會被位在粒腺體內膜的branched-chain alpha-keto acid dehydrogenase complex(BCKDC)代謝成MCPA。這個BCKDC酵素有異常，產生的疾病就是大名鼎鼎「楓糖尿症」(Maple Syrup Urine Disease, MSUD)。MCPA會亂搜刮身體的輔酶A(Coenzyme A, CoA)和肉鹼(carnitine)。前者可說是我們身體在降階營養素必備的物質；後者是我們體內天然的解毒劑與長鏈脂肪酸進入細胞的鑰匙，於是這些孩童的重要分子就被用盡了。

同時，MCPA會抑制所有的acyl CoA dehydrogenase(醯基輔酶A脫氫酶，見圖)。真核生物有九種醯基輔酶A脫氫酶，五種和短、中、長與極長鏈脂肪酸代謝有關；四種分別代謝白胺酸(leucine)、異白胺酸(isoleucine)、纈胺酸(valine)與離(lysine)/色胺酸(tryptophan)。代謝的基本ABC概念就是，這些脂肪酸、胺基酸、糖類只要沒辦法順利走完變成二氧化碳、水、(尿素)，中間的產物就會毒害人體。這當中最毒的就是白胺酸和離/色胺酸的中介產物，叫做異戊酸和戊二酸。這兩個疾病代謝不能分別叫做惡名昭彰的「異戊酸血症」(Isovaleric acidemia, IVA)與「戊二酸血症第一型」(Glutaric acidemia,GA-1)是台灣新生兒篩檢指定項目，所有在我國出生的新生兒一定會做到這兩種疾病的篩檢。這兩種毒性物質過高(可以中和毒性的肉鹼也沒了)會對腦部產生不可逆的傷害(還會散發出惡臭)，同時，耗盡的葡萄糖無法透過脂肪酸與胺基酸的代謝(糖質新生仰賴的輔酶NADH與脂肪酸/部份胺基酸代謝後的乙醯輔酶A都耗竭了)而回補。根據這篇文獻，大流行時每天都有5位左右兒童因為嚴重腦病變過世，嚴重的病人就會同時表現IVA/GA-1的臨床徵象，好似真正得到這種先天代謝疾病，渾身抽筋痛苦而死，非常類似台北榮總準備要直接以基因篩檢的「戊二酸血症第二型」(GA-2，或稱作MADD)，解毒妙方就是造成不可逆的傷害前給予超高劑量(300倍於一般建議攝取量)的維生素B2(核黃素)+肉鹼(你平常吃維生素錠後尿尿會黃黃的就是因為B2吸收光譜導致的啦)，以及及時矯正血糖。

一段生 #荔枝 中毒的新聞，居然涉及到如此深奧的代謝學問。

#我覺得MCPA太毒了還是不要拿來當降血糖藥好惹。

👉 Demystifying 𝗠𝘆𝘁𝗵 𝘃𝘀 𝗙𝗮𝗰𝘁 about SUGAR: It's fine to add a teaspoon of sugar or a teaspoon of honey, or a teaspoon of maple syrup. That's not the problem.⠀
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It's the 34 teaspoons we're eating every day, day in and day out.⠀
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First of all, the science is beyond question. Sugar in all its forms is the root cause of our obesity epidemic and most of the chronic disease sucking the life out of our citizens and our economy — and, increasingly, the rest of the world. ⠀
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You name it, it’s caused by sugar: heart disease, cancer, dementia, type 2 diabetes, depression, and even acne, infertility and impotence.⠀
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Being addicted to sugar and flour is not an emotional eating disorder. ⠀
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It’s a biological disorder, driven by hormones and neurotransmitters that fuel sugar and carb cravings — leading to uncontrolled overeating. This is not a limited phenomenon. It’s the reason nearly 70 percent of Americans and 40 percent of kids are overweight. It's almost similar in Malaysia, too
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You can break your addiction by:⠀
- Eating real food⠀
- Starting your day with a nutritious breakfast⠀
- Reducing your stress⠀
- Exercising regularly⠀
- Getting good quality sleep⠀
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Join my upcoming 👉 #3in1Transformation program to learn more about healthy foods and weight management. Click the link in my Instagram profile @doctors.bulletin or whatsapp me from my Facebook page DR. SIMON MSH

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