200423955 玖、發明說明： 【發明所屬之技術領域】 本發明有關一種肌肉營養不良之治療劑及治療用食品。更詳言 之，係有關一種含有榖胺酸胜肽之肌肉營養不良治療劑及肌肉營養不 良之治療用食品及使用該等治療劑或治療用食品之治療方法。 【先前技術】 肌肉營養不良為肌肉組織因變性及壞死所漸進產生之以肌肉力降 低、肌肉萎縮為主要症狀之遺傳性疾病之總稱，依據遺傳形式，可分 類為X染色體性劣性之裘馨氏(Duchenne)型及貝克氏(Becker)型營養 不良；正常染色體之性劣性之四肢軀幹型、先天性、遠位型營養不良； 正常染色體之性優性之顏面肩胛上腕型、肌肉緊張型營養不良。 其中，以裘馨氏型、四肢軀幹型、顏面肩胛上腕型佔患者中之大 部分，以裘馨氏型為最多，過去，係以裘馨氏型為中心進行研究。 裘馨氏型營養不良之患者大多為男性，於幼兒期發症，一旦發症 其發展停頓而束手無策，經常為發展性地使肌肉組織可能經過脂肪與 結締組織間之置換’而在11歲前後無法站立，且因為心臟不全、呼吸 不全等併發症而平均在20歲左右之年齡死亡。 該等裘馨氏型營養不良係由於在X染色體(Xp2U)中存在有肌縮 蛋白运傳基因缺陷，而無法生產肌縮蛋白（dyStr〇phin)之蛋白質因此 而發症已至為清楚。由於肌縮蛋白遺傳基因為由乃個以上之基因外顯 子(exon)所構成之約2, 300kb之龐大基因，因此遺傳基因之缺陷率高，、 裘馨氏(Duchenne)型肌肉營養不良症已提高至家族病史之非突然變異 患者數之約1/3。該肌縮蛋白遺傳基因之遺傳基因產物之肌縮蛋白之蛋 白質係存在於肌肉細胞之形質膜正下方，而與肌肉顧維之構成蛋白 質之肌動蛋白及形質膜之肌縮蛋白結合糖蛋白質結合。 雖然嘗試於肌肉投予插入該等肌縮蛋白遺傳^因之 之f叙雜基目躲，財好__短，ί 再投予效果不佳利題，至目前為止尚無充分之治療法。 又’關於其他病型，依據種種蛋白質缺陷或異常，雖然已漸明瞭 IP040077/SF-1037f 6 200423955 引起該疾病之原因，但目前尚未有明確的有效治療劑及治療方法。 至於肌肉營養不良之漸進抑制劑，於日本特開平8一198756號公報 中，曾提出一種特徵為含有海草之細胞膜成分之角叉聚糖 (carrageenan)之肌肉營養不良之漸進抑制劑。 然而，該等肌肉營養不良之漸進抑制劑對老鼠、鷄等動物進行投 予試驗’只不過確認動物之肌肉細胞肥大或增殖，而對人類之肌肉營 養不良之漸進抑制效果尚未明。 【發明内容】 本發明者基於上述情況進行刻意研究之結果，發現藉由使用L一榖 胺酸含量高之榖胺酸胜肽，可抑制人類肌肉營養不良之漸進，而可改 善其症狀，因而完成本發明。 本發明之目的在於提供一種可抑制肌肉營養不良之漸進、可改盖 其症狀之治療劑及治療用食品。又，本發明提供一種使用含有榖胺酸 胜肽之治療劑及/或治療用食品治療肌肉營養不良之方法。 亦即，本發明有關一種肌肉營養不良之治療劑，其特徵為 胺酸胜肽。 匁救 又，本發明又有關一種肌肉營養不良之治療劑，其特徵為含 胺酸胜肽及泛醌。 又 本發明有關一種肌肉營養不良之治療用食品，其特徵為含有經添 加之威胺酸胜肽作為活性成分，較好又含有泛酿。 前述之榖胺酸胜肽較好為L-榖胺酸含量為15至6〇質量％且依據凝 膠過濾法所測定之平均分子量為2〇〇至1〇〇,〇〇〇之胜肽，又期望為來 自小麥穀蛋白者。 又’前述之泛醌較好為辅酵素Q10。 本發明有關一種肌肉營養不良之治療方法，其特徵為使用前述之 肌肉營養不良治療劑或肌肉營養不良之治療用食品。 【實施方式】 以下，具體說明本發明。 本發明有關一種肌肉營養不良之治療劑，其特徵為含有榖胺酸胜 EP040077/SF-1037f 7 200423955 肽。 肽中&紐酸含4高之胜肤。若雜胺酸胜 天然蛋白質中調製。因此制’但超過60質姆難以自 該榖胺酸胜im姻^4^1調製之容易性及經濟性方面， 較好為2G至40㈣ 為G f恤下，通常為15至⑼㈣， 定到嶋伽法所測 异出者，或榖胺酸胜肽為合成物之愔 二里為基準所 酸使用比例而求得。 4 '、 口成0^所用之1^麵胺 虱之千衡’明顯可看出具有抗潰瘍效果、創傷治瘢效 識到作為輸入液、經腸營養劑之重要性。 而重新_ 本發明中所用之榖胺酸胜肽之平均分子量 ，，較好為5。。至20, _之範圍，更好為^⑽0二= 圍。前述之榖職胜肽之平均分子量若小於2 二二2 道不良之傾向，另-方面，若榖胺酿肽之平均S3,而有味 時，添加水時會形成黏稠結塊，而此操作性劣化里二⑽’_ 胺酸胜肽之平均分子#触據郷賴顧崎時之人=之榖 本發明中所用之榖胺酸胜肽可為天然或重纽 =刀子篁。 分解，依據化學合成或遺傳基心程方法 加水 製造方法並無特別關，而可_習知方t，、心所製造時，其 此情況下，由原料獲得之容易性、穩定供應性 觀之，以使用為天然蛋白質之小麥榖蛋白較有利。 双牛等觀點 小麥榖蛋白主要為麥谷蛋白與麥醇溶蛋白所構成之蛋入 物’因為含多量之L-楚胺酸作為構成胺基酸，因此由 ^二 水分解，比較容易製造L-麩胺酸含量高之胜肽。 請白之加 8 IP040077/SF-1037f 200423955 亦即，小麥榖蛋白通常含有25至50質量—麵胺酸作為構成胺 土酸，為獲得平均分子量在2GG至100, GGG範_之如此條件，可依 據蛋白酵素、酸、驗等使小麥榖蛋白加水分解，並視需要依據劃分等 之^行，比較容易獲得L-麩胺酸含量在15至6〇質量%範圍内之榖胺酸 胜肽。 該情況下，賴之小麥榖蛋自亦可使耻生的榖蛋自狀態下予以 j化後，進-步使該轉榖蛋白預先赠、齡靴學處理或以酵 f等，予生物處理所得之分子量降低者或提高與蛋_素之親合 之小麥榖蛋白。 體言之’可以以曰本特開昭64一47353號公報、特許第298193 μ、觸平5-2獅9號公報所揭示之方法製造榖胺酸胜肤。 例如，較好使用例如依據日本特開昭64—概3號公報之揭示，使 ^白酶與其他之雜蛋自酶之複合蛋_目定純錄珠粒等擔體 上，而處理小麥榖蛋白之方法，或 δ 公鄉2985193射齡之持榖蛋自蛋白酶或澱 i白酶、Ιϊίΐίΐ。該方法所用之蛋白酶可舉例如胃蛋白酶、胰 _叔75 /蛋白酶、紅絲且Μ⑽7麵)起源之蛋白酶、曲 屬起源之蛋自酶、木瓜蛋自酶、錄蛋自酶等之各種蛋白酶。 份有勝™ ^_峨GM (日清藥業股 等榖胺酸胜肽亦可施以加水分解、劃分等處理。 據而要該 酸二肌肉營養不良之治療劑’除了前述之榖胺 料、之重要湘子，為存在於如真核缝之細胞線 錢之辅二為輔帽，而具有下式構造之n=6 Ο200423955 (1) Description of the invention: [Technical field to which the invention belongs] The present invention relates to a therapeutic agent and therapeutic food for muscular dystrophy. More specifically, the present invention relates to a therapeutic agent for muscular dystrophy containing muscle peptides, a therapeutic food for muscular dystrophy, and a therapeutic method using the therapeutic agent or therapeutic food. [Previous technology] Muscular dystrophy is a general term for genetic diseases with muscle strength reduction and muscle atrophy that are gradually produced by degeneration and necrosis of muscle tissue. According to the genetic form, it can be classified as X-chromosome inferiority. (Duchenne) type and Becker's type of malnutrition; sexually inferior limbs of normal chromosomes, trunk type, congenital, and distant type malnutrition; sexually superior features of normal chromosomes, scapular upper wrist type, muscular tension type malnutrition. Among them, Qiu Xin's type, limb trunk type, facial scapular upper wrist type accounted for most of the patients. Qiu Xin's type was the most. In the past, the research was based on Qiu Xin's type. Most patients with Qiu Xin's type of malnutrition are males, who develop symptoms in early childhood. Once they develop symptoms, their development stops and they are helpless. They often develop muscles that may undergo replacement between fat and connective tissue. He was unable to stand, and died at an average age of about 20 years due to complications such as cardiac insufficiency and respiratory insufficiency. Due to the deficiency of the myosin transporter gene in the X chromosome (Xp2U), the Qiuxin-type malnutrition is unable to produce the protein of dystropin, so the onset of the disease is clear. The gene of dystrophin is a huge gene of about 2,300 kb, which is composed of more than one gene exon, so the defect rate of the genetic gene is high. Duchenne muscular dystrophy has been Increased to about 1/3 of the number of patients with non-abrupt mutations in family history. The myosin protein of the gene gene product of the myosin genetic gene exists directly below the plasma membrane of muscle cells, and binds to the actin constituting protein of muscle Gu Wei and the myosin-binding glycoprotein of the plasma membrane. . Although attempts have been made to insert these genes into the muscles, it is necessary to avoid the fs and miscellaneous subjects. The wealth is good and short, and the re-administration is not effective. So far, there is no sufficient treatment. Regarding other disease types, although the cause of the disease has been gradually clarified based on various protein defects or abnormalities, IP040077 / SF-1037f 6 200423955 has not yet clearly defined effective therapeutic agents and treatment methods. As a progressive inhibitor of muscular dystrophy, Japanese Patent Laid-Open No. 8-198756 has proposed a progressive inhibitor of muscular dystrophy characterized by carrageenan containing a cell membrane component of seaweed. However, these progressive inhibitors of muscular dystrophy have been administered to animals such as mice and chickens', but they have only confirmed the hypertrophy or proliferation of muscle cells in animals, and the effect of progressive suppression of muscular dystrophy in humans has not yet been determined. [Summary of the Invention] As a result of deliberate research based on the above situation, the present inventors found that by using a peptide having a high content of L-pyramic acid, it can inhibit the progression of human muscular dystrophy and improve its symptoms. The present invention has been completed. The object of the present invention is to provide a therapeutic agent and a therapeutic food which can inhibit the progression of muscular dystrophy and can cover its symptoms. The present invention also provides a method for treating muscular dystrophy by using a therapeutic agent and / or a therapeutic food containing a glutamic acid peptide. That is, the present invention relates to a therapeutic agent for muscular dystrophy, which is characterized by an amino acid peptide. Rescue In addition, the present invention also relates to a therapeutic agent for muscular dystrophy, which is characterized in that it contains urethane peptide and ubiquinone. The present invention also relates to a food for treating muscular dystrophy, which is characterized in that it contains added peptidyl peptide as an active ingredient, and preferably contains pan-fermented. The amidine peptide is preferably a peptide having an L-amylate content of 15 to 60% by mass and an average molecular weight of 200 to 100,000 measured by a gel filtration method, It is also expected to be from wheat gluten. The aforementioned ubiquinone is preferably coenzyme Q10. The present invention relates to a method for treating muscular dystrophy, which comprises using the aforementioned therapeutic agent for muscular dystrophy or a therapeutic food for muscular dystrophy. [Embodiment] Hereinafter, the present invention will be specifically described. The present invention relates to a therapeutic agent for muscular dystrophy, which is characterized in that it contains amidin EP040077 / SF-1037f 7 200423955 peptide. Peptide & neoic acid contains 4 high levels of skin. Wakame is better than natural protein. Therefore, it is difficult to make it more than 60 masses from the ease and economics of the amino acid ^ 4 ^ 1 modulation, preferably 2G to 40. For G-shirts, usually 15 to ⑼㈣, set to Calculated by the method of stigma or the ratio of acid used based on the glutamic acid peptide as the base of the compound. 4 ', mouth into 0 ^ 1 ^ facial amines used in the thousand balance' can obviously be seen with anti-ulcer effect, wound healing scar effect recognize the importance of as an input solution, enteral nutrition. However, the average molecular weight of the ammonium peptide used in the present invention is preferably 5. . The range to 20, _, more preferably ^ ⑽0 二 = 围. If the average molecular weight of the aforesaid peptides is less than 2-22, it tends to be bad. On the other hand, if the average S3 of the amidine peptide is odorous, it will form a sticky cake when adding water, and this operation The average molecular weight of the diaminopyrene's amino acid peptides in the deterioration is as follows: The person who uses the amino acid peptides in the present invention can be natural or heavy = knives. Decomposition, the method of adding water based on chemical synthesis or genetic basis path method is not particularly relevant, but can be obtained from raw materials in terms of the ease of supply and stable supply. It is more advantageous to use wheat prion protein as a natural protein. Shuang Niu and other viewpoints Wheat gluten protein is mainly an egg product composed of glutenin and gliadin. 'Because it contains a large amount of L-chuamine acid as a constituent amino acid, it is decomposed by ^ dihydrate, and it is easier to produce L. -High peptide with glutamic acid. Please add the white 8 IP040077 / SF-1037f 200423955 That is, wheat peptone protein usually contains 25 to 50 mass-glutamic acid as a constituent amino acid, in order to obtain such conditions that the average molecular weight is 2GG to 100, GGG range_ The wheat peptone protein is hydrolyzed according to protease, acid, and test, and if necessary, according to the division, etc., it is relatively easy to obtain peptidyl peptides having an L-glutamic acid content in the range of 15 to 60% by mass. In this case, Lai's wheat eggs can also be transformed into jelly eggs in a state, and then the transgenic protein can be donated in advance, treated with aging boots, or treated with yeast f. The reduced molecular weight may increase the wheat prion protein which has affinity for egg white. In a word, glutamic acid can be produced by the methods disclosed in Japanese Patent Application Laid-Open No. Sho 64-47353, Patent No. 298193 μ, and Touching No. 5-2 Shio 9. For example, it is preferred to treat wheat gluten by using, for example, a compound egg such as white enzyme and other enzymes from the disclosed in Japanese Patent Application Laid-Open No. 64-3, to treat pure wheat beads. The method of protein, or δ Gongxiang 2985193 shoot-aged eggs from protease or lyase, Ιϊίΐίΐ. The protease used in this method includes, for example, various proteases such as pepsin, pancreatic acid / protease, red silk, and M⑽7) origin protein, Aspergillus egg self-enzyme, papaya egg self-enzyme, egg record self-enzyme and the like.有 有 胜 ™ ^ _ EGM (Nissin Pharmaceutical Co., Ltd. glutamate peptides can also be treated with hydrolysis, classification and so on. According to the treatment of the acid two muscle dystrophy 'in addition to the aforementioned amine materials The most important Xiangzi is the auxiliary cap which exists in the cell line money such as eukaryotic sutures, and has the following structure: n = 6 Ο
IP040077/SF-1037f 200423955 其令’本發明中較妤使用n=l〇之辅酵素qi〇。 上述中本發明有關之肌肉營養不良之治療劑雖以含有前述榖胺酸 胜肽之特徵為第一樣態及除了該榖胺酸胜肽以外又含有泛醌之特徵為 第二樣態，但前者之情況下，亦可採用不含有泛醌之肌肉營養不良之 治療劑與泛醌分別投予之方式使用。 本發明之肌肉營養不良之治療劑僅含有榖胺酸胜肽作為活性成 分，可採用對應於投予路徑之任意劑型。 亦即’本發明有關之肌肉營養不良之治療劑劑型，可使用例如鍵 劑、散劑、顆粒劑、膠囊劑、糖漿劑、乾糖漿齊卜溶液劑、口含片等 d型。其中，通常使用種種添加劑，例如賦型劑、黏合劑、安定劑、 ^劑、分制、稀_、界面活性劑、緩衝劑、甜味劑、著色劑、 轉等’依據—般方法製造而得。但是，由於辅酵飾為 性’而可溶解於植物性油、動物性油等非親水性之有機 制祕自紐鮮财麟齡分散及 米左右或以下程度之微鱗Γ。之吸高’亦可制平均粒徑在1微 油、=言用之添加劑可舉例如，例如大豆油、紅花 二t甘=:醇等之多價醇;山梨酸__、斧 製 山梨糖醇液、糖漿等賦型劑；甜味=醇㈣1月曰、阿拉伯膠、 再者，液體製劑在服用時可為^^1、邱調節劑、香料等。 中的形式。又，鍵劑、顆其他適當溶劑 易地攝取有效成分。換言之 ^^可含於食品中，因而可更容 製造本發明之肌肉營養不良之二中添加上述之榖胺酸胜肽’而 加上述之泛Si。_是_^ 食品。再者，期望於該食品中添 至於該等食品，可舉例如流°體食物、湯類、果汁類、乳品飲料、 IP040077/SF-1037f 10 200423955 類型。該等食品爾健康食品、機能:食=5=等 每日投予量進行管理。 ’較好以 但亦 又，液 心又、^液Ϊ食品之情況下’可由最初之液狀食品加以調製， 可由粉末或糊狀加以調製，亦可溶解於特定量之水性液體 狀食品為展現各種風味可添加各種添加劑，例如，較好添加展 安定劑 2劑等。亦可進-步齡各種營養素、各種軸命、義質、食物 纖、准、多價不飽和脂肪轉之其他營養素、分散劑、乳化劑、 甜味劑等。 本發明之肌肉營養不良之治療方法之槪為使时叙肌肉營養 不良治療劑及/或肌肉營養不良之治療財品，該治療舰/或該食品 可對肌肉營養不良之患者經口或經腸投予或攝取。 又，兩述威胺酸胜肽為得自小麥榖蛋白之安全性高之物質，而泛 醌尤其是辅酵素Q!。為近年來廣用作為健康食品之安全性高之物質。 此時，榖胺酸胜肽之投予量係隨病症、用法、年齡、體重、性別 等而異，可依此適當加以決定，但在成人之情況下，投予範圍通常為i 至40克/曰，較好為5至10克/曰之範圍。 又，除了榖胺酸胜肽以外，併用泛醌之情況下，泛醌之投予量雖 與榖胺酸胜肽之投予量一起決定，但期望在成人之情況下，投予量範 圍通常為5至200毫克/曰，較好為1〇至1〇〇毫克/曰之範圍。 一般，肌肉營養不良之診斷係依據發症年齡、初發症狀、家族史 等，進行血液及尿物之生物化學檢查、肌肉CT、肌肉電圖、生物檢測 肌肉之組織學以及生物化學檢查等所進行者。 其中，患者血液之生物化學檢查可看出源自肌酸鱗酸激酶(CPK)、 醛縮酶、乳糖脫氫酶(LDH)、榖胺酸-草醯乙酸轉澱粉酶(GOT)、麩胺酸 -丙酮酸轉澱粉酶(GPT)等之肌肉形質（肌襞）之酵素血中濃度增加，以 及肌肉組織中作為氧儲存體之機能之肌紅蛋白之血中濃度增加。尤其 是，裘馨氏(Duchenne)型肌肉營養不良者，其肌酸構酸激酶(CPK)之血 11 IP040077/SF-1037f 200423955 中濃度顯著增加。 至於其中之原因’ }日出除了肌肉組織崩壞、壞死以外，還加上肌 肉細胞膜之透過性異常亢進。 因而對心者之血液進行定期的生物化學檢查，可依據所測得之 血中源自肌肉形質(肌漿)之酵素以及肌紅蛋白之濃度，作為肌肉營養 不良進行情況之生物化學指標之一。 又’本發明之肌肉營養不良之治療劑、治療用食品當然亦可對人 類以外之動物投予，該等適用並無任何除外。 發明效果 ' 本發明之肌肉營養不良之治療劑及肌肉營養不良之治療用食品以 及使用該等治麵及治療时品之治射法，可抑制肌崎養不良的罾 進行，並可改善症狀等。又，本發明中所用之榖胺酸胜肽、辅酵素如 之安全性優異，目此雜糾卩撕予。據此，本發明之治療劑及治療 用之食品在治療肌肉營養不良方面極有用。 實施例 以下基於實施例具體說明本發明，但本發明不限定於該等實施例。 又，實施例中，榖胺酸胜肽之平均分子量及該胜肽中L一榖胺酸之 含量以下列方法測定。 〈胜肽之平均分子量〉 胜肽之水溶液經0· 45微米之膜過濾器過濾，測定凝膠過濾後之分鲁 子量。管柱使用日本BioRad實驗室公司製之rBiosil SEC125—5」。測 定條件為測定波長為280nm，溶離液為〇.2m μ磷酸緩衝液(p册.〇， 、 〇· 1%SDS)，流速為0· 2毫升/分鐘。又，使用卵氫蛋白（分子量44kDa)、 肌球蛋白（分子量17kDa)以及維他命B12(分子量1· 35kDa)作為分子量 - 之標準。 〈胜肽中L-榖胺酸含量〉 含有醯胺態氮之L-胺基酸雖然有榖胺酸及天門冬胺酸兩種，但小 麥蛋白質中所含之含有醯胺態氮之胺基酸中有95質量％以上為榖胺 酸。亦即，小麥榖蛋白中含有醯胺態氮之胺基酸量就此作為L-榖胺酸 IP040077/SF-1037f 12 200423955 因而，小麥榖蛋白t之含醯胺態氮就此 之含量’而不會有太大誤差 表不為L-麵胺酸之含量。 百先，榖胺酸胜肽中所含之醒胺態氮之含量係由·〇χ之化學物 質士中醯胺定量法[Meth· En獅!，n，36—65⑽7)]求得。具體言之， 將榖胺酸胜肽置入C_y之燒杯中，於其中添加m鹽酸;使^胺酸 胜肽中之_態氮游離為氨，所發生之氨依據衛生檢查指針[日本藥學 曰，ΐ之衛生試驗法註解」，第274一276頁，金原出版社(1"0)]予、 以定量，由所定量之氨量求得榖胺酸胜肽中所含之醯胺態氮量。 基於所得之醯胺態氮量，算出榖胺酸胜肽中含有酸胺態氮之胺基 酸含量，該值作為L-榖胺酸含量。 製造例1榖胺酸胜肽之製造 鲁 ⑴於反應爸中-齊置入離子交換水9,700克、檸檬酸肝38公斤 以及小麥榖蛋白（活性榖蛋白，West〇n食品股份有限公司製）丨乃⑼公IP040077 / SF-1037f 200423955 which makes the use of a coenzyme qi of n = 1 in the present invention. Although the above-mentioned therapeutic agent for muscular dystrophy related to the present invention is characterized by containing the aforementioned glutamic acid peptide as the first state and characterized by containing the ubiquinone peptide as the second state, In the former case, a therapeutic agent for muscular dystrophy that does not contain ubiquinone and ubiquinone may be administered separately. The therapeutic agent for muscular dystrophy of the present invention contains only the amino acid peptide as an active ingredient, and any dosage form corresponding to the administration route can be adopted. That is, the therapeutic dosage form of the muscular dystrophy related to the present invention can be used in the form of type d such as a bonding agent, a powder, a granule, a capsule, a syrup, a dry syrup solution, a buccal tablet, and the like. Among them, various additives are generally used, such as excipients, adhesives, stabilizers, additives, fractions, diluents, surfactants, buffers, sweeteners, colorants, transfer agents, etc. Got. However, because co-fermentation is sexual, it can be dissolved in non-hydrophilic organic ingredients, such as vegetable oils and animal oils. It can also be used to make an average particle size of 1 micro-oil. = Additives that can be used include, for example, polyvalent alcohols such as soybean oil and safflower di-titanium: alcohol; sorbic acid, sorbose Excipients such as alcohol solution and syrup; sweetness = alcohol, January, Arabic gum, and more, the liquid preparation can be ^^ 1, Qiu regulator, flavor, etc. when taking. In the form. In addition, the bonding agent and other appropriate solvents can easily ingest active ingredients. In other words, ^^ can be contained in food, so it can be more suitable to add the above-mentioned glutamic acid peptide 'and the above-mentioned pan-Si to the second muscular dystrophy of the present invention. _YES_ ^ Food. Furthermore, it is desirable to add such foods to the food, for example, fluid foods, soups, juices, dairy drinks, IP040077 / SF-1037f 10 200423955. These foods are healthy food, function: food = 5 =, etc. The daily dosage is managed. 'Preferably but also in the case of liquid core and liquid food' can be prepared from the original liquid food, can be prepared from powder or paste, or can be dissolved in a certain amount of liquid food. Various flavors can be added with various additives, for example, two stabilizers and the like are preferably added. It can also be used as a nutrient, a variety of nutrients, various nutrients, food fiber, quasi, polyvalent unsaturated fats and other nutrients, dispersants, emulsifiers, sweeteners and so on. One of the methods for treating muscular dystrophy of the present invention is to make a therapeutic agent for muscular dystrophy and / or a therapeutic product for muscular dystrophy. The therapeutic vessel and / or the food can be administered to patients with muscular dystrophy by mouth or intestines. Administration or ingestion. In addition, the two peptides are a highly safe substance derived from wheat prion protein, and ubiquinone is especially a coenzyme Q !. It has been widely used in recent years as a highly safe substance for health foods. At this time, the administration amount of glutamic acid peptide varies with the disease, usage, age, weight, sex, etc., and can be appropriately determined according to this, but in the case of an adult, the administration range is usually i to 40 g The range is preferably 5 to 10 g / day. In addition, when ubiquinone is used in addition to the ammonium peptide, the amount of ubiquinone to be administered is determined together with the amount of ammonium peptide, but it is expected that in the case of an adult, the range of the amount of administration is usually It is in the range of 5 to 200 mg / day, preferably 10 to 100 mg / day. Generally, the diagnosis of muscular dystrophy is based on the age of onset, onset symptoms, family history, etc., blood and urine biochemical examinations, muscle CT, electromyography, histology of bioassay muscles, and biochemical examinations. Proceeder. Among them, the biochemical examination of the patient's blood showed that it originated from creatine squamate kinase (CPK), aldolase, lactose dehydrogenase (LDH), ammonium-oxaloacetate transamylase (GOT), and glutamine. Acid-pyruvate transamylase (GPT) and other muscle form (myosin) enzymes increase in blood concentration, and muscle tissues function as oxygen storage function of myoglobin blood concentration. In particular, those with Duchenne muscular dystrophy have significantly increased blood levels of creatine kinase (CPK) 11 IP040077 / SF-1037f 200423955. As for the reason ’,} In addition to the breakdown and necrosis of muscle tissue, there is also an abnormally high permeability of the muscle cell membrane. Therefore, regular biochemical examination of the heart's blood can be used as one of the biochemical indicators of the progress of muscular dystrophy based on the measured blood-derived enzymes and myoglobin concentration in the blood. . It is needless to say that the therapeutic agent and therapeutic food for muscular dystrophy of the present invention can also be administered to animals other than humans, and these applications are not excluded. Effects of the Invention 'The therapeutic agent for muscular dystrophy and therapeutic food for muscular dystrophy according to the present invention, and the method of treating radiating muscles using these treatments and treatment products can suppress the progression of muscular dystrophy and improve symptoms. . In addition, the ammonium peptides and coenzymes used in the present invention are excellent in safety, and are therefore miscellaneous. Accordingly, the therapeutic agent and therapeutic food of the present invention are extremely useful in treating muscular dystrophy. Examples The present invention will be specifically described below based on examples, but the present invention is not limited to these examples. In the examples, the average molecular weight of the ammonium peptide and the content of L-amylate in the peptide were measured by the following methods. <Average molecular weight of peptide> The aqueous solution of the peptide was filtered through a 0.45 micron membrane filter, and the fraction after gel filtration was measured. As the column, rBiosil SEC125-5 manufactured by BioRad Laboratories, Japan was used. The measurement conditions were a measurement wavelength of 280 nm, an eluent of 0.2 m μ phosphate buffer (p.0.0, 0.1% SDS), and a flow rate of 0.2 ml / min. In addition, egg hydrogen protein (molecular weight of 44 kDa), myosin (molecular weight of 17 kDa), and vitamin B12 (molecular weight of 1.35 kDa) were used as standards for molecular weight-. <L-Amino Acid Content in Peptide> Although there are two kinds of L-amino acids containing amine nitrogen, amines containing asparagine and aspartic acid, but the amine group containing amine nitrogen is contained in wheat protein 95% by mass or more of the acid is amidine. That is to say, the amount of amino acids containing amidine nitrogen in wheat prion protein is thus taken as the L-amylate IP040077 / SF-1037f 12 200423955. Therefore, the content of amidine nitrogen in wheat peptone t is not such a content. There is too much error to indicate the content of L-plane amino acid. Baixian, the content of ammonium nitrogen in the ammonium peptide is determined by the quantitative method of amidine in the chemical chemist [Meth · En lion !, n, 36-65⑽7)]. Specifically, the ammonium peptide was placed in a beaker of C_y, and m hydrochloric acid was added thereto; the _-state nitrogen in the ammonium peptide was freed to ammonia, and the ammonia that occurred was based on the health inspection guidelines [Japanese Pharmaceutical Daily "Annotation of Sanitary Test Method", pp. 274-276, Kanehara Publishing House (1 " 0)], Quantitatively, the ammonium nitrogen contained in the ammonium peptide is obtained from the quantified amount of ammonia. the amount. Based on the obtained ammonium nitrogen content, the amino acid content of the ammonium peptide containing the ammonium nitrogen was calculated, and this value was used as the L-ammonium acid content. Production Example 1 Production of amidin peptides in the reaction paw-9,700 g of ion-exchanged water, 38 kg of citrate liver, and wheat prion protein (active prion protein, manufactured by West On Food Co., Ltd.) Nagano
斤，加溫至45°C後，添加蛋白酶（天野製藥株式會社製之「蛋白質MAfter heating to 45 ° C, add protease ("Protein M by Amano Pharmaceutical Co., Ltd.
Amano」）2· 2公斤及澱粉酶（阪急生物工業股份有限公司製之「液態酵 素T」）1·1么斤，於45 c進行加水分解歷時5小時，接著使用25質 量％之氫氧化鈉溶液，使溶液之ρΗ調整至4· 5至4· 5維持5小時進行 酵素處理。 。（2)接著，使溶液在80°C保持20分鐘使蛋白酶失活後，冷卻至65 c，於其中添加澱粉驗(阪急生物工業股份有限公司製之「液態酵素τ」）馨 0.5公斤，使小麥榖蛋白中所含之澱粉質及纖維質加水分解後，在^ C保持20分鐘使殺粉酶失活。 (3) 接著，溶液冷卻至1〇t以下後，再度加熱至阳它，於其中添 加活性碳（武田藥品工業股份有限公司製之rTakec〇n」）1〇〇公^；，二· 55°C攪拌30分鐘。 ' (4) 溶液溫度在45°C下，添加過濾助劑（昭和化學工業股份有限公 司製之「RachioRite」），使用加壓過濾裝置進行過濾，回收渡液7 〇⑽ 公升(7 m3)。 C5)於上述(4)中回收之濾液以Brix值為2〇至4〇之下減壓濃縮 IP040077/SF-1037f 13 200423955 後，使用加熱板在ll〇°C加熱2〇秒進行殺菌，接著冷卻至诏。^。 (6)上述(5)所得之溶液，使用喷霧乾燥裝置以送風溫度l6〇t、排 風溫度之餅τ儒觀，麟小錄蛋自之桃分解物之 酸胜肽粉末1，000公斤。 σ)上述(6)所得之榖胺酸胜肽粉末使用60網目之網篩（網目開孔 〇.246麵)予以分級，回收可通過60網目網篩之微粉末(榖胺酸胜肤微 粉）0Amano ") 2.2 kg and amylase (" liquid enzyme T "manufactured by Hankyu Bio-Industry Co., Ltd.) 1.1 kg, hydrolyzed at 45 c for 5 hours, and then 25% by mass sodium hydroxide was used Solution, adjust the pH of the solution to 4.5 to 4 and 5 for 5 hours for enzyme treatment. . (2) Next, the solution was kept at 80 ° C for 20 minutes to inactivate the protease, and then cooled to 65 c, and 0.5 kg of starch ("Liquid Enzyme τ" manufactured by Hankyu Bio-Industry Co., Ltd.) was added thereto to make After hydrolyzing the starch and cellulite contained in wheat peptone, it was kept at ^ C for 20 minutes to inactivate the dusting enzyme. (3) Next, after the solution was cooled to 10 t or less, it was heated to yang again, and activated carbon (rTakecON "manufactured by Takeda Pharmaceutical Industry Co., Ltd.) was added to it. C for 30 minutes. '(4) Add a filtration aid (“RachioRite” manufactured by Showa Chemical Industry Co., Ltd.) at a solution temperature of 45 ° C, and use a pressure filtration device to perform filtration to recover 70 liters (7 m3) of liquid. C5) The filtrate recovered in the above (4) was concentrated under reduced pressure IP040077 / SF-1037f 13 200423955 under a Brix value of 20 to 40, and then sterilized by heating at 110 ° C for 20 seconds using a hot plate, and then Cool to 诏. ^. (6) The solution obtained in the above (5), using a spray-drying device, at a supply air temperature of 160t and an exhaust air temperature of the cake τ ruguan, Lin Xiaolu egg from the peach decomposition product of acid peptide powder 1,000 kg . σ) The carbamic acid peptide powder obtained in the above (6) was classified using a 60-mesh mesh sieve (open-mesh 0.246 side), and the fine powder that can pass through the 60-mesh mesh sieve (pyridine peptide) was recovered. 0
(8)上述(7)中所回收之榖胺酸胜肽(微粉)測定其平均分子量及卜 榖胺酸含量，獲得平均分子量約為MGG及卜榖胺酸含量約32質量％。 製造例2至4榖胺酸胜肽之製造 如衣k例1所述之相同方法，獲得下表1所示性質之榖胺酸胜肽。 表1 平均分子量 L-榖胺酸含量 製造例2 300 25質量％ 製造例3 10, 000 32質量％ 製造例4 16,000 34質量％ 實施例1錠劑之製造 製造例1所獲得之榖胺酸胜肽83· 3克、結晶纖維素(旭化成股份 有限公司）10克以及聚乙烯吡咯烷酮(BASF公司製)5克予以混合，於其 中添加乙醇30毫升，依據濕式法依據一般方法製造顆粒。依此所得之肇 顆粒乾燥後，添加硬脂酸鎂L 2克作為打錠顆粒粉末，使用打鍵機打 鍵’製造每錠1克之錠劑1〇〇個(相當於錠劑每一錠之榖胺酸胜肽含量 〇· 838 克）。 實施例2糖漿劑之製造 ‘ 精製水400克予以煮沸，於其中添加混合之白糖75〇克以及製造 例2所得之榖胺酸胜肽1〇〇克並予以溶解，趁熱時以布過濾，於其中 添加精製水至總量1000毫升製造糖漿劑(相當於糖漿劑1〇〇毫升含有 10克穀胺酸胜肽）。 實施例3顆粒劑之製造 IP040077/SF-1037f 14 200423955 榖胺酸胜肽GP-1 (平均分子量7,麵，l—榖胺酸含量32質量％ ;曰 清藥業股份有限公司製）76克、乳糖(DMV公司製）13 3夯、社晶纖維 細化姐份有_) 6· 7克以及聚乙職 克予以混合’於其巾添加30毫升乙醇，依據赋法絲—般方法製造 顆粒，乾燥後，獲得整粒之顆粒劑(相冑於顆粒劑101之穀胺酸胜肽 含量為7.6克）。 實施例4流體食物之製造 . 於約65°C純水中，添加酪蛋白鈉(DMV公司製）4〇克、麥芽糖糊精 (三和澱粉公司製)160克以及榖胺酸胜肽GfHl(曰清藥業股份有限公司 製)25克並予以溶解，接著添加維他命混合物5克及微量礦物質之各成 分混合液。該混合物置入均質混合機(特殊機械化工業製），以約8〇〇〇響 rpm粗乳化15分鐘。所得乳化液冷卻至約2〇它，添加香料後，進行最 終混合。該乳液以母份230克充填入小袋中，一邊進行氣氣置換一邊 密封該小袋，在m°c進行15分鐘殺g獲得翻之越食物。該流體 食物相當於每230克中榖胺酸胜肽含量約5克。 實施例5麵包之製造 小麥粉（咼筋麵粉)150克與乾燥酵母2克予以混合。此時另外將榖 胺&L胜妝GP-1 (曰清樂業股份有限公司製）2〇克、砂糖2〇克、食鹽3 克、脫脂奶粉6克溶於溫開水70克，添加雞蛋一個予以充分混合。添 加於前述小麥粉中，以手充分揉合後，添加奶油4〇克充分揉合，製作泰 20個生麵包捲。接著經發酵後，於其表面上塗抹蛋漿，於18(rc烤箱 中烘烤約15分鐘，製造麵包捲。該麵包捲相當於每個含有榖胺酸胜肽 約1克。 實施例6義大利麵條用肉醬之製造 · 一人份之義大利麵條用肉醬（15〇克）置入锅内，同時添加榖胺酸胜 肽GP-1(曰清藥業股份有限公司製）5克及輔酵素q1q(日清藥業股份有 限公司製）30毫克，在肉醬一邊溫熱狀態下溶解硬膠囊，作成義大利 麵條用肉醬。該肉醬充填入小袋後，一邊進行氮氣置換一邊密封該小 袋’在121°C進行15分鐘殺菌獲得含有榖胺酸胜肽及辅酵素q10之義大 IP040077/SF-1037f 15 200423955 利麵條用肉醬。 實施例7烏龍麵之製造 榖胺酸胜肽GP-1(曰清藥業股份有限公司製）20克及補 ❽。（曰 清藥業股份有限公司製）400毫克預先予以混合。相對於小麥♦分筋 麵粉）400克，於水200克中添加前述之穀胺酸胜肽及補酵素&。之混 合物，分散食鹽20克，充分混合放置隔夜。隨後，生枒料予以=伸\ 切斷成寬約5毫米製造烏龍麵。於沸騰開水中烹煮約1〇分鐘，其外觀、 味道及食感均良好。該等烏龍麵相當於一人份食物含有^ f 胜肽及約100毫克辅酵素Q10。 、a兄敦欣 試驗例1 <症狀例1 ;裘馨氏型進行性肌肉營養不良症（男性，4歲)> 依據-歲過制始學步行_料_，兩 ，，小之階梯，三歲時期大約踏開腳步但手若未扶著絲法站立； 展現Go·徵候群，而診斷為裘馨氏型進行性肌肉營養不良症。 豐血液檢查後’開始對病患以榖胺酸胜肽GM(日清藥 =2質後文源自、蛋白平均分子量7, 〇°〇，L" 榖胺酸； 篁為以貝;£/6 ’後文中亦同）每曰9去 η Α 2克之罝混合入優酪乳或牛奶中經口 投予，自開始投予後經過L 5個月進行 結果示於表2。 一 表2(8) The average molecular weight and content of the amino acid peptide (micro powder) recovered in the above (7) were measured, and the average molecular weight was about MGG and the content of the amino acid was about 32% by mass. Production Examples 2 to 4 Production of glutamic acid peptides The glutamic acid peptides having the properties shown in Table 1 below were obtained in the same manner as described in Example 1. Table 1 Average molecular weight L-Larmic acid content Manufacturing Example 2 300 25% by mass Manufacturing Example 3 10,000 32% by mass Manufacturing Example 4 16,000 34% by mass Manufacturing Example 1 Lozenges obtained in Manufacturing Example 1 Peptide 83.3 g, 10 g of crystalline cellulose (Asahi Kasei Co., Ltd.) and 5 g of polyvinylpyrrolidone (manufactured by BASF) were mixed, 30 ml of ethanol was added thereto, and granules were produced according to a general method by a wet method. After the obtained Zhaozhao granules were dried, 2 g of magnesium stearate L was added as the granule powder for granulation, and a key press was used to manufacture 100 granules of 1 g per granule (corresponding to amidine of each granule). Acid peptide content (.838 g). Example 2 Production of syrup 400 g of purified water was boiled, and 75 g of mixed white sugar and 100 g of glutamic acid peptide obtained in Production Example 2 were added and dissolved, and filtered while hot with a cloth. To this was added purified water to a total amount of 1000 ml to make a syrup (equivalent to 100 ml of syrup containing 10 g of glutamic acid peptide). Example 3 Manufacture of granules IP040077 / SF-1037f 14 200423955 Phenylaminopeptide GP-1 (average molecular weight 7, noodles, l-pyridine content 32% by mass; Yueqing Pharmaceutical Co., Ltd.) 76 g , Lactose (produced by DMV) 13 3 tamping, shejing fiber refinement _) 6.7 grams and polyethyl ethoxylate were mixed '30 ml of ethanol was added to the towel, and granules were made according to the method of Fufa silk- After drying, whole granules are obtained (the content of glutamate peptides in granules 101 is 7.6 g). Example 4 Manufacture of fluid food. In pure water at about 65 ° C, 40 g of sodium caseinate (manufactured by DMV), 160 g of maltodextrin (manufactured by Sanwa Starch Co., Ltd.) and glutamic acid peptide GfHl ( 25 g of Yueqing Pharmaceutical Co., Ltd.) was dissolved, and then 5 g of a vitamin mixture and a mixed solution of each component of trace minerals were added. This mixture was placed in a homomixer (manufactured by Special Mechanized Industries), and coarsely emulsified at about 8000 rpm for 15 minutes. The obtained emulsion was cooled to about 20, and after adding the fragrance, it was finally mixed. This emulsion was filled into a pouch with 230 g of the mother portion, and the pouch was sealed while gas-gas replacement was performed, and then killed at 15 ° C for 15 minutes to obtain food. This fluid food is equivalent to about 5 grams of glutamic acid peptide per 230 grams. Example 5 Production of Bread 150 g of wheat flour (gluten flour) and 2 g of dry yeast were mixed. At this time, 20 g of amidine & L Shengzhuang GP-1 (manufactured by Qingle Industry Co., Ltd.), 20 g of sugar, 3 g of salt, 6 g of skimmed milk powder were dissolved in 70 g of warm water, and eggs were added. One is thoroughly mixed. It was added to the wheat flour, kneaded by hand, and 40 grams of cream was added to knead it to make 20 Thai bread rolls. After fermentation, apply egg syrup on the surface, and bake in a 15 ° C oven for about 15 minutes to make a bread roll. This bread roll is equivalent to about 1 g of each of the amino acid peptides. Example 6 Manufacture of spaghetti noodles · One serving of spaghetti noodles (150 g) is placed in the pot, and 5 g of glutamic acid peptide GP-1 (manufactured by Qing Pharmaceutical Co., Ltd.) is added, and Coenzyme q1q (manufactured by Nissin Pharmaceutical Co., Ltd.) 30 mg, dissolve hard capsules in the meat sauce while warming it, and make it into a spaghetti noodle sauce. After filling the meat sauce in a sachet, it is sealed while being replaced with nitrogen This pouch 'was sterilized at 121 ° C for 15 minutes to obtain Yida IP040077 / SF-1037f 15 200423955, a meat sauce for noodles, containing glutamine peptide and coenzyme q10. Example 7 Production of glutamine peptide from udon noodles GP-1 (Yueqing Pharmaceutical Co., Ltd.) 20 grams and supplements. (Yueqing Pharmaceutical Co., Ltd.) 400 mg are mixed in advance. Compared to wheat ♦ gluten flour 400 grams, 200 grams of water The aforementioned glutamate peptide and supplement enzyme & are added. The mixture was dispersed in 20 g of common salt and thoroughly mixed overnight. Subsequently, the raw materials were stretched and cut into 5mm wide to make udon noodles. After boiling in boiling water for about 10 minutes, its appearance, taste and texture are good. These udon noodles are equivalent to one serving of food containing ^ f peptide and about 100 mg of coenzyme Q10. A, Dunxin Test Example 1 < Symptom Example 1; Qiu Xin's progressive muscular dystrophy (male, 4 years old) > Based on the age-based system to learn to walk _ feed_, two, small steps At the age of three, he stepped forward but stood with his hands unsupported; showing Go syndrome, and was diagnosed with Qiu Xin's progressive muscular dystrophy. After the blood test, the patient began to take glutamic acid peptide GM (Nisshin Yasu = 2 quality sources, and the average molecular weight of the protein was 7, 〇 ° 〇, L " glutamic acid; 篁 to shellfish; £ / 6 'The same applies hereinafter) 9 to η Α 2 grams of 罝 罝 mixed into yogurt or milk and orally administered. The results are shown in Table 2 after 5 months from the start of administration. A table 2
投予1.5個月後 16,830 293 514 1,177 BP040077/SF-1037f 16 200423955 醛縮酶（IU/升） (基準值；1.7〜5. 7) 177.5 112 肌紅蛋白（奈克/毫升） (基準值；60以下） 790 720 由表2可看出，血液中之CPK、G0T、GPT、LDH、縮醛酶及肌紅蛋 白之值，與開始投予前相較，各值均降低而判斷症狀獲得改善。 又’自投予開始後3週後，手不用扶著即可站立而爬上高π公分 之階梯’隨後，可爬上高低差小之階梯，接著腳可站立而開始走路。 試驗例2 〈症狀例2 ;顏面肩胛上腕型肌肉營養不良症（男性，⑼歲)〉 自20歲左右開始顯現左上腕倦怠感，3〇歲左右左肩舉起困難，隨 後’頸部'兩肩、兩上肢感到肌肉無力以及走路後下肢感到肌肉無力 等症狀顯著，45歲時診斷為顏面肩胛上腕型肌肉營養不良症。自5〇歲 左右，左肩完全難以舉起，並發展至步行障礙之階段。 最近，對被判定為翼狀肩胛、因顏面肌肉之肌肉無力感無法吹口 哨之呈現表情憂鬱之患者實施血液檢查後，開始經口投予榖胺酸胜肽 GP-1(9克/天），自開始投予後經過3個月進行血液檢查。 結果不於表3。 表3 投予前 投予3個月後 CPK(IU/升） (基準值；35〜200) 245 141 肌紅蛋白（奈克/毫升） (基準值；60以下） 95 61 由表3可看出，血中之CPK、肌紅蛋白值，與投予前相較，各均降 低而判斷症狀獲得改善。又，自開始投予經過3個月後，頸部及上肢 之肌肉無力感以及步行障礙獲得減輕。 試驗例3 〈症狀例3 ;裘馨氏形進行性肌肉營養不良（男性，6歲)> 17 IP040077/SF-1037f 200423955 對自兩歲左右出現難以站立，近來踏開腳步但手若未扶著床無法 站立所展現Gowers徵候群且兩側下腿肌肉有假性肥大、可片刻站:搖 晃步行之患者實施血液檢查後，開始經口投予榖胺酸胜肽(3克/ 天)及泛S昆(辅酵素Q10 ;曰清藥業股份有限公司，以下同）洲毫克/天， 自開始投予後經過3個月後，又自開始投予經過6個月後，進行血 檢查。 結果示於表4。 表4 CPK(IU/升） (基準值；35〜200) 投予前 16, 868 投予3個月後 13, 589 投予後6個& 1,465 LDH(U) (基準值；140〜430) 2,169 1,124 1,112 肌紅蛋白（奈克/毫升） (基準值；60以下） 960 540 480 由表4可看出，血中之CPK、LDH、肌紅蛋白值，與投予前相較， 各均降低而判斷症狀獲得改善。又，自開始投予經過6個月後，患者 容易站立，而可以小走程度蹣跚步行。 試驗例4 〈症狀例4 ;肌肉緊張性營養不良症（男性，26歲)> # 自22歲左右兩手之握力降低且全身倦怠，而被診斷為肌肉緊張性 營養不良症之患者，針對肌肉緊張每曰投予米歐納(My〇nal)(R)(鹽酸愛 普松(eperisone hydrochloride))進行物理 匕學療法。 對該患者實施血液檢查後，開始經口投予榖胺酸胜肽Gp—1(8克/ · 天)及泛Sbb(輔酵素Q10) 90毫克/天，自開始投予後經過g個月後，又 自開始投予經過6個月後，進行血液檢查。 又，剛開始投予時，患者兩手之握力為右手5公斤，左手5 5公 斤，具有全身倦怠、舌或大拇指球之扣診肌肉強直病、手握緊後不容 易鬆弛之抓握肌肉強直症之症狀。 IP040077/SF-1037f 18 200423955 結果示於表5。1.5 months after administration 16,830 293 514 1,177 BP040077 / SF-1037f 16 200423955 Aldolase (IU / liter) (reference value; 1.7 ~ 5.7) 177.5 112 Myoglobin (neck / ml) (reference value; Below 60) 790 720 As can be seen from Table 2, the values of CPK, GOT, GPT, LDH, plasmalogen, and myoglobin in the blood are lower than those before administration, and the values are judged to be improved. . Also, “3 weeks after the start of the administration, the hand can stand without holding up and climb up to a height of π cm” Then, you can climb up the step with a small height difference, and then stand with your feet to start walking. Test example 2 <Symptom example 2; Facial scapular upper-wrist muscular dystrophy (male, younger age)> The left upper wrist burnout began to appear around the age of 20, and the left shoulder was difficult to lift around the age of 30, and then the 'neck' shoulders 2. The symptoms of muscle weakness in the upper limbs and muscle weakness in the lower limbs after walking were significant. At the age of 45, he was diagnosed with facial scapular and wrist-type muscular dystrophy. Since the age of 50, her left shoulder has been difficult to lift, and she has developed into a walking disorder. Recently, a blood test was performed on a patient who was judged to be a pteroscapular scapula, who was unable to whistle due to the muscle weakness of the facial muscles, and then started oral administration of Peptide GP-1 (9 g / day) Blood tests were performed 3 months after the start of administration. The results are not in Table 3. Table 3 CPK (IU / liter) 3 months before administration (reference value; 35 to 200) 245 141 Myoglobin (neck / ml) (reference value; 60 or less) 95 61 The values of CPK and myoglobin in the blood were lower than those before administration, and it was judged that the symptoms were improved. In addition, three months after the start of administration, the muscle weakness of the neck and upper limbs and walking disorders were alleviated. Test Example 3 <Symptom Example 3; Qiu Xin's progressive muscular dystrophy (male, 6 years old) > 17 IP040077 / SF-1037f 200423955 For those who have difficulty standing since the age of two, they have recently stepped away but their hands are not supported The Gowers syndrome manifested by immobilization and immobilization on the lower leg muscles on both sides can stand for a moment: after shaking and walking, a blood test was started, and oral peptides (3 g / day) and pan-peptide were started. S Kun (Coenzyme Q10; Yueqing Pharmaceutical Co., Ltd., the same below) Zhou mg / day, 3 months after the start of administration, and 6 months after the start of administration, blood tests were performed. The results are shown in Table 4. Table 4 CPK (IU / liter) (base value; 35 ~ 200) 16, 868 before the investment 3, 589 3 months after the investment 13, 589 6 after the investment & 1,465 LDH (U) (base value; 140 ~ 430) 2,169 1,124 1,112 Myoglobin (nike / ml) (reference value; below 60) 960 540 480 As can be seen from Table 4, the values of CPK, LDH, and myoglobin in the blood were all compared with those before administration. Decrease and judge improvement of symptoms. In addition, after 6 months from the start of the administration, the patient was easy to stand, and could walk with a small walk. Test Example 4 <Symptom Example 4; Muscular tonic dystrophy (male, 26 years old)> # Patients diagnosed with muscular tonic dystrophy have reduced grip strength in both hands and general burnout since around the age of 22 Anxiety was given to Myonal (R) (eperisone hydrochloride) for physical therapy. After a blood test was performed on this patient, oral administration of glutamic acid peptide Gp-1 (8 g / day) and pan-Sbb (coenzyme Q10) 90 mg / day was started, and g months after the start of administration After 6 months from the start of administration, blood tests were performed. Also, at the beginning of administration, the patient's grip in both hands was 5 kg in the right hand and 55 kg in the left hand. He had general burnout, tongue or thumb ball buckling, diagnosed muscle rigidity, and grip muscle rigidity that was not easy to relax after gripping. Symptoms. IP040077 / SF-1037f 18 200423955 The results are shown in Table 5.
表5 〈症狀例5 ;遠位型營養不良症（男性，7〇歲)> 在35歲左右被診斷為肌肉營養不良症，約15年前兩手兩腳之肌 肉力量降低，最近被判定握力降低(右手12公斤，左手13公斤）以及 四肢遠位肌肖麟魏，而有站立步娜礙H實施錢檢查後， 開始經口投予榖胺酸胜月太Gp一ι(8克/天)及泛醌(辦素_) 9〇毫克/ 天，自開始投予後經過3個月後，又自開始投予經過6個月後，進行 血液檢查。 結果不於表6。 表6 投予前 投予3個月後 投予後6個月 CPKCIU/升） (基準值；35〜200) 251 149 148 醛縮酶（IU/升） (基準值；1· 2〜7. 6) 5· 7 4.2 4.2 肌紅蛋白（奈克/毫升） 170 120 79 IP040077/SF-1037f 19 200423955 以下） 由表6可看出’血中之QPJ[、酸縮酶、肌紅蛋白值，與投予前相較， 各均降低而判斷症狀獲得改善。又，自開始投予經過6個月後，站立 後可蹣跚步行，四肢倦怠感獲得改善，手的握力大幅升高(右手20公 斤，左手21公斤）。 產業利用性 本發明之肌肉營養不良之治療劑、肌肉營養不良之治療用食品， 由於可抑制人狀肌㈣養不良之發展，可改善錄，^在醫藥 康食品、機能性食品、醫療用食品等領域具有利用性。 【圖式簡單說明】 【圖式代表符號說明】 20 IP040077/SF-1037fTable 5 <Symptom Example 5; Dystrophy (male, 70 years old)> was diagnosed as muscular dystrophy at about 35 years old, and the muscle strength of both hands and feet decreased about 15 years ago, and it was recently judged that the grip strength Lowered (12 kg in the right hand, 13 kg in the left hand) and Xiao Lin Wei, a distant muscle of the extremities, and after having performed a money check, he began to orally administer glutamic acid Katsukita Gp (8 g / day) ) And ubiquinone (bansu_) 90 mg / day, 3 months after the start of administration, and 6 months after the start of administration, blood tests were performed. The results are not shown in Table 6. Table 6 CPKCIU / liter 6 months after administration 3 months before administration (reference value; 35 ~ 200) 251 149 148 Aldolase (IU / liter) (reference value; 1.2 · 7.6 ) 5 · 7 4.2 4.2 Myoglobin (neck / ml) 170 120 79 IP040077 / SF-1037f 19 200423955 or less) From Table 6, it can be seen that 'QPJ [, acid condensation enzyme, myoglobin value in blood, and Compared with the pre-administration, each of them decreased and it was judged that the symptoms were improved. In addition, after 6 months from the start of administration, she can walk stably after standing, her limbs burnout improved, and her grip strength increased significantly (20 kg for her right hand and 21 kg for her left hand). INDUSTRIAL APPLICABILITY The therapeutic agent for muscular dystrophy and the therapeutic food for muscular dystrophy of the present invention can inhibit the development of human muscular dystrophy and improve the recording. ^ In medicine, health food, functional food, medical food And other fields are useful. [Schematic description] [Schematic symbol description] 20 IP040077 / SF-1037f